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ALL cures require many MRD therapies. This strategy should drive experiments and trials in metastatic bone sarcomas.

The surgical management of extremity bone and soft tissue sarcomas has evolved significantly over the last 50 years. The introduction and refinement of high-resolution cross-sectional imaging has allowed accurate assessment of anatomy and tumor extent, and in the current era more than 90% of patients can successfully undergo limb-salvage surgery. Advances in imaging have also revolutionized the clinician's ability to assess treatment response, detect metastatic disease, and perform intraoperative surgical navigation. This review summarizes the broad and essential role radiology plays in caring for sarcoma patients from diagnosis to post-treatment surveillance. Present evidence-based imaging paradigms are highlighted along with key future directions., (© 2024. The Author(s), under exclusive licence to International Skeletal Society (ISS).)

Introduction: The decision to treat metastatic bone disease (MBD) surgically depends in part on patient life expectancy. We are unaware of an international analysis of how life expectancy among these patients has changed over time. Therefore, we asked (1) how has the life expectancy for patients treated for MBD changed over time, and (2) which, if any, of the common primary cancer types are associated with longer survival after treatment of MBD?, Methods: We reviewed data collected from 2000 to 2022 in an international MBD database, as well as data used for survival model validation. We included 3,353 adults who underwent surgery and/or radiation. No patients were excluded. Patients were grouped by treatment date into period 1 (2000 to 2009), period 2 (2010 to 2019), and period 3 (2020 to 2022). Cumulative survival was portrayed using Kaplan-Meier curves; log-rank tests were used to determine significance at P < 0.05. Subgroup analyses by primary cancer diagnosis were performed., Results: Median survival in period 2 was longer than in period 1 ( P < 0.001). Median survival (at which point 50% of patients survived) had not been reached for period 3. Median survival was longer in period 2 for all cancer types ( P < 0.001) except thyroid. Only lung cancer reached median survival in period 3, which was longer compared with periods 1 and 2 ( P < 0.001). Slow-growth, moderate-growth, and rapid-growth tumors all demonstrated longer median survival from period 1 to period 2; only rapid-growth tumors reached median survival for period 3, which was longer compared with periods 1 and 2 ( P < 0.001)., Discussion: Median duration of survival after treatment of MBD has increased, which was a consistent finding in nearly all cancer types. Longer survival is likely attributable to improvements in both medical and surgical treatments. As life expectancy for patients with MBD increases, surgical methods should be selected with this in mind., Level of Evidence: VI., (Copyright © 2024 by the American Academy of Orthopaedic Surgeons.)

Percent necrosis (PN) following chemotherapy is a prognostic factor for survival in osteosarcoma. Pathologists estimate PN by calculating tumor viability over an average of whole-slide images (WSIs). This non-standardized, labor-intensive process requires specialized training and has high interobserver variability. Therefore, we aimed to develop a machine-learning model capable of calculating PN in osteosarcoma with similar accuracy to that of a musculoskeletal pathologist. In this proof-of-concept study, we retrospectively obtained six WSIs from two patients with conventional osteosarcomas. A weakly supervised learning model was trained by using coarse and incomplete annotations of viable tumor, necrotic tumor, and nontumor tissue in WSIs. Weakly supervised learning refers to processes capable of creating predictive models on the basis of partially and imprecisely annotated data. Once "trained," the model segmented areas of tissue and determined PN of the same six WSIs. To assess model fidelity, the pathologist also estimated PN of each WSI, and we compared the estimates using Pearson's correlation and mean absolute error (MAE). MAE was 15% over the six samples, and 6.4% when an outlier was removed, for which the model inaccurately labeled cartilaginous tissue. The model and pathologist estimates were strongly, positively correlated (r = 0.85). Thus, we created and trained a weakly supervised machine learning model to segment viable tumor, necrotic tumor, and nontumor and to calculate PN with accuracy similar to that of a musculoskeletal pathologist. We expect improvement can be achieved by annotating cartilaginous and other mesenchymal tissue for better representation of the histological heterogeneity in osteosarcoma., (© 2023 Orthopaedic Research Society.)

Chondromyxoid fibroma is a rare, benign tumor of the bone with excellent prognosis but a high rate of recurrence. We report a patient presenting with pain and a history of chondromyxoid fibroma of the distal left femur previously treated with multiple prior curettage and bone graft procedures. Magnetic resonance imaging and histopathology indicated a recurrence of tumor. Due to the small size of the tumor recurrence and challenges associated with prior open surgery, the patient underwent cryoablation of the lesion with computed tomography guidance. Follow-up 18 months later indicated a resolution of pain and improvement on magnetic resonance imaging, and no concerns after 20 months. To our knowledge, this is the first reported case of chondromyxoid fibroma treated with cryoablation. This case suggests cryoablation could be considered in the setting of recurrent chondromyxoid fibroma for local tumor control., (© 2023. The Author(s), under exclusive licence to International Skeletal Society (ISS).)

Background and Objectives: Distinguishing sarcomatoid carcinoma from primary sarcoma is clinically important. We sought to characterize metastatic sarcomatoid bone disease and its management., Methods: We analyzed the characteristics of all cases of sarcomatoid carcinoma to bone at a single institution from 2001 to 2021, excluding patients with nonosseous metastases. Survival was evaluated using the Kaplan-Meier method., Results: We identified 15 cases of metastatic sarcomatoid carcinoma to bone. In seven cases the primary cancer was unknown at presentation. Renal cell carcinoma was suspected or confirmed in nine cases. Nine patients presented with pathologic fracture and two with concomitant visceral metastases. All patients underwent image-guided core needle or open biopsy. Ten required surgery for discrete osseous metastases; in four cases definitive surgery was delayed (median delay, 19 days) due to inability to rule out sarcoma with frozen section. No patients required reoperation or had construct failure. Thirteen died of disease; median survival was 17.5 months (interquartile range, 6.2-25.1)., Conclusions: Metastatic sarcomatoid carcinoma is a clinically challenging entity. Multidisciplinary input and communication are key to identifying the primary carcinoma, locating osseous metastases, and defining an operative fixation that will survive the remainder of the patient's life., (© 2023 Wiley Periodicals LLC.)

A subset of clear cell chondrosarcomas may contain focal areas of low-grade conventional chondrosarcoma; however, it is rare to find foci resembling clear cell chondrosarcoma admixed with areas otherwise typical conventional chondrosarcoma. We report two patients with conventional chondrosarcoma with clear cell features occurring in the rib, one in the setting of multiple hereditary exostoses (MHE) and the other without MHE. Both patients were found to have a destructive rib mass with a soft tissue component and underwent en bloc resection. Histologic examination revealed predominantly grade 2 conventional chondrosarcomas; however, multiple foci containing large cells with pale eosinophilic to clear cytoplasm, distinct cell borders, centrally located nuclei, and conspicuous nucleoli, resembling clear cell chondrosarcoma were identified throughout the specimen. The significance of clear cell features in an otherwise typical conventional chondrosarcoma, to our knowledge, is unknown and deserves recognition. Finally, these tumors highlight the need for careful histologic examination and proper classification as unexpected findings may impact management.

Ewing sarcomas (ES) are rare small round cell sarcomas often affecting children and characterized by gene fusions involving one member of the FET family of genes (usually EWSR1) and a member of the ETS family of transcription factors (usually FLI1 or ERG ). The detection of EWSR1 rearrangements has important diagnostic value. Here, we conducted a retrospective review of 218 consecutive pediatric ES at diagnosis and found eight patients having data from chromosome analysis, FISH/microarray, and gene-fusion assay. Three of these eight ES had novel complex/cryptic EWSR1 rearrangements/fusions by chromosome analysis. One case had a t(9;11;22)(q22;q24;q12) three-way translocation involving EWSR1::FLI1 fusion and 1q jumping translocation. Two cases had cryptic EWSR1 rearrangements/fusions, including one case with a cryptic t(4;11;22)(q35;q24;q12) three-way translocation involving EWSR1::FLI1 fusion, and the other had a cryptic EWSR1::ERG rearrangement/fusion on an abnormal chromosome 22. All patients in this study had various aneuploidies with a gain of chromosome 8 (75%), the most common, followed by a gain of chromosomes 20 (50%) and 4 (37.5%), respectively. Recognition of complex and/or cryptic EWSR1 gene rearrangements/fusions and other chromosome abnormalities (such as jumping translocation and aneuploidies) using a combination of various genetic methods is important for accurate diagnosis, prognosis, and treatment outcomes of pediatric ES.

Background: 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., Methods: 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., Results: 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., Conclusions: 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., (© 2022 American Cancer Society.)

Ollier disease (OD) and Maffucci Syndrome (MS) are rare disorders characterized by multiple enchondromas, commonly causing bone deformities, limb length discrepancies, and pathological fractures. MS is distinguished from OD by the development of vascular anomalies. Both disorders are cancer predisposition syndromes with malignancies developing in ~50% of the individuals with OD or MS. Somatic gain-of-function variants in IDH1 and IDH2 have been described in the enchondromas, vascular anomalies and chondrosarcomas of approximately 80% of the individuals with OD and MS. To date, however, no investigation of germline causative variants for these diseases has been comprehensively performed. To search for germline causative variants, we performed whole exome sequencing or whole genome sequencing of blood or saliva DNA in 94 unrelated probands (68 trios). We found that 7 had rare germline missense variants in HIF1A, 6 had rare germline missense variants in VHL, and 3 had IDH1 variants including 2 with mosaic IDH1-p.Arg132His variant. A burden analysis using 94 probands assigned as cases and 2,054 unrelated individuals presenting no OD- or MS-related features as controls, found that variants in HIF1A, VHL, and IDH1 were all significantly enriched in cases compared to controls. To further investigate the role of HIF-1 pathway in the pathogenesis of OD and MS, we performed RNA sequencing of fibroblasts from 4 probands with OD or MS at normoxia and at hypoxia. When cultured in hypoxic conditions, both proband and control cells showed altered expression of a subset of HIF-1 regulated genes. However, the set of differentially expressed genes in proband fibroblasts included a significantly reduced number of HIF-1 regulated genes compared to controls. Our findings suggest that germline or early post-zygotic variants identified in HIF1A, VHL, and IDH1 in probands with OD and MS underlie the development of the phenotypic abnormalities in a subset of individuals with OD and MS, but extensive functional studies are needed to further confirm it., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2022 Poll et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Purpose: Novel effective therapies are urgently needed in recurrent osteosarcoma. GD2 is expressed in human osteosarcoma tumours and cell lines. This study evaluated the disease control rate (DCR) in patients with recurrent osteosarcoma treated with the anti-GD2 antibody dinutuximab plus cytokine therapy as compared to historical outcomes., Methods: AOST1421 was a single-arm Phase 2 study for patients with recurrent pulmonary osteosarcoma in complete surgical remission. Patients received up to five cycles of dinutuximab (70 mg/m 2 /cycle) with granulocyte-macrophage colony-stimulating factor (GM-CSF). Two different dinutuximab infusion schedules were studied: 35 mg/m 2 /day over 20 h (2 days) and 17.5 mg/m 2 /day over 10 h (4 days). Primary end point was DCR, defined as a proportion of patients event free at 12 months from enrolment. The historical benchmark was 12-month DCR of 20% (95% CI 10-34%). Dinutuximab would be considered effective if ≥ 16/39 patients remained event free. Secondary objectives included toxicity evaluation and pharmacokinetics., Results: Thirty-nine eligible patients were included in the outcome analysis. Dinutuximab did not demonstrate evidence of efficacy as 11/39 patients remained event free for a DCR of 28.2% (95% CI 15-44.9%). One of 136 administered therapy cycles met criteria for unacceptable toxicity when a patient experienced sudden death of unknown cause. Other ≥ Grade 3 toxicities included pain, diarrhoea, hypoxia, and hypotension. Pharmacokinetic parameters were similar in the two schedules., Conclusions: The combination of dinutuximab with GM-CSF did not significantly improve DCR in recurrent osteosarcoma. Dinutuximab toxicity and pharmacokinetics in adolescent and young adult osteosarcoma patients were similar to younger patients. Other strategies for targeting GD2 in osteosarcoma are being developed., Competing Interests: Conflict of interest statement The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: SGD has received consulting fees from Amgen, Bayer, and Jazz as well as travel expenses from Loxo, Roche, and Salarius. MDK has received consulting fees from Merck, Sharpe and Dhome. KAJ has received consulting fees from Bayer and Ipsen and honoraria from Takeda and Foundation Medicine. The remaining authors have no conflicts of interest., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

The anatomic extent of a pelvic bone tumor and the need for reconstruction dictate the type of pelvic resection (limb salvage pelvic resection or amputation). If a pelvic bone tumor resection involves two or more critical anatomic structures (the sciatic nerve, femoral neurovascular bundle or the hip joint), then reasonable functional recovery after limb salvage is less likely and amputation should be considered. Both limb salvage and amputation approaches to the pelvis are technically arduous surgeries with significant associated morbidity and complications. As such, imaging plays an important role in the post-operative management of patients who have undergone pelvic bone tumor resection. In this article, we will review optimal imaging techniques as well as the expected post-operative appearance after pelvic bone tumor resection and important complications including infection, tumor recurrence, and complications related to complex soft tissue and osseous reconstruction.

Background: Current therapy for osteosarcoma pulmonary metastases (PMs) is ineffective. The mechanisms that prevent successful immunotherapy in osteosarcoma are incompletely understood. We investigated the tumor microenvironment of metastatic osteosarcoma with the goal of harnessing the immune system as a therapeutic strategy., Methods: 66 osteosarcoma tissue specimens were analyzed by immunohistochemistry (IHC) and immune markers were digitally quantified. Tumor-infiltrating lymphocytes (TILs) from 25 specimens were profiled by functional cytometry. Comparative transcriptomic studies of distinct tumor-normal lung 'PM interface' and 'PM interior' regions from 16 PMs were performed. Clinical follow-up (median 24 months) was available from resection., Results: IHC revealed a statistically significantly higher concentration of TILs expressing immune checkpoint and immunoregulatory molecules in PMs compared with primary bone tumors (including programmed cell death 1 (PD-1), programmed death ligand 1 (PD-L1), lymphocyte-activation gene 3 (LAG-3), T-cell immunoglobulin and mucin domain-containing protein 3 (TIM-3), and indoleamine 2,3-dioxygenase (IDO1). Remarkably, these lymphocytes are excluded at the PM interface compared with PM interior. TILs from PMs exhibited significantly higher amounts of PD-1 and LAG-3 and functional cytokines including interferon-γ (IFNγ) by flow cytometry. Gene expression profiling further confirmed the presence of CD8 and CD4 lymphocytes concentrated at the PM interface, along with upregulation of immunoregulatory molecules and IFNγ-driven genes in the same region. We further discovered a strong alternatively activated macrophage signature throughout the entire PMs along with a polymorphonuclear myeloid-derived suppressor cell signature focused at the PM interface. Expression of PD-L1, LAG-3, and colony-stimulating factor 1 receptor (CSF1R) at the PM interface was associated with significantly worse progression-free survival (PFS), while gene sets indicative of productive T cell immune responses (CD8 T cells, T cell survival, and major histocompatibility complex class 1 expression) were associated with significantly improved PFS., Conclusions: Osteosarcoma PMs exhibit immune exclusion characterized by the accumulation of TILs at the PM interface. These TILs produce effector cytokines, suggesting their capability of activation and recognition of tumor antigens. Our findings suggest cooperative immunosuppressive mechanisms in osteosarcoma PMs including immune checkpoint molecule expression and the presence of immunosuppressive myeloid cells. We identify cellular and molecular signatures that are associated with patient outcomes, which could be exploited for successful immunotherapy., Competing Interests: Competing interests: GC is an employee/paid consultant for Compugen LTD. DMC reports grant support from AstraZeneca and Rainier Pharmaceuticals. DMP reports grant support from Bristol Myers Squibb., (© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Chondroblastoma is a rare benign tumor of immature cartilage cells that generally occurs in an epiphyseal location of skeletally immature individuals. However, a few studies have reported cases in older patients. The purpose of this study was to evaluate the clinical, radiographic, and pathologic features of chondroblastoma in an adult population. The pathology archives of our institution were searched for cases of chondroblastoma diagnosed in patients ≥25 years of age. Of 14 patients identified, 8 were male and 6 were female with a median age of 34 years (range = 29-54 years). Most lesions occurred in short bones of hands and feet (N = 7, 50%), followed by the long tubular bones (N = 4, 28%). All demonstrated typical histologic features of chondroblastoma, but more extensive calcification, necrosis, and degenerative changes were also seen. At follow-up (median = 73.5 months), 2 patients (17%) had local recurrence. None had metastasis. In summary, chondroblastoma in adults tends to involve the short bones of the hands and feet and demonstrate histologic changes associated with long-standing growth of a benign tumor.

Pelvic bone tumors present a diagnostic and therapeutic challenge. Due to the deep anatomic location and resultant late clinical presentation, pelvic bone tumors tend to be large and located in close proximity to pelvic viscera as well as vital neurovascular structures. Operative management of pelvic bone tumors is indicated for a variety of orthopedic oncologic conditions. In general, limb-sparing pelvic resection rather than hemipelvectomy with amputation of the ipsilateral limb is considered when a functional limb can be preserved without compromising the surgical margins. There are various options for pelvic resection and reconstruction, and the selection depends on tumor histology, anatomic location, and extent. The decision regarding choice of surgical procedure and reconstruction method for a pelvic bone tumor requires a thorough knowledge of the pelvic anatomy, and careful inspection of the anatomic extent. The surgical plan must strike a balance between acceptable functional outcome and acceptable morbidity. In this review, we describe the different types of pelvic resection techniques, and the vital role preoperative imaging plays in defining the anatomic extent of a pelvic bone tumor and subsequent surgical planning.

Introduction: The goals of orthopaedic treatment for most patients with osseous metastases are to control pain, maintain function, and maximize quality of life and time at home. The aim of this study was to determine differences in 30-day postoperative morbidity and mortality between patients who underwent prophylactic versus postfracture stabilization for metastatic lesions of long bones., Methods: The American College of Surgeons National Surgical Quality Improvement Program database was queried for patients who underwent prophylactic fixation (n = 461) or postfracture stabilization (n = 856) for pathologic fractures because of metastatic lesions of long bones from 2006 to 2016. The groups were compared with respect to several potential confounders using Student t, Kruskal-Wallis, and χ tests. Logistic and Poisson regression models (inclusion threshold of P < 0.1) were used to assess the associations of functional status with outcomes. The alpha level was set at 0.05., Results: Prophylactic fixation was associated with a lower risk of major medical complications (odds ratio = 0.64; 95% confidence interval [CI], 0.45 to 0.93; P = 0.02), discharge to a care facility rather than home (odds ratio = 0.48; 95% CI, 0.36 to 0.63; P < 0.01), and lower risk of a longer hospital stay (incidence risk ratio = 0.86; 95% CI, 0.74 to 0.96; P = 0.01) compared with postfracture stabilization. No significant difference was found in the risk of unplanned revision surgery or 30-day postoperative mortality between the two groups., Conclusion: Although prevention of pathologic fractures caused by metastatic disease may not always be possible, patients who underwent prophylactic stabilization had a lower risk of major complications within 30 days postoperatively and shorter hospital stays compared with patients who underwent postfracture stabilization., Level of Evidence: Level IV, retrospective cohort.

Osseous metastases to the hands and feet (acrometastases) are exceedingly rare. Historically, the most common primary cancer observed has been lung carcinoma, treated either nonoperatively or with amputation. The aim of this study was to describe the clinical characteristics associated with acrometastases. This was a retrospective review of 28 patients with histologically proven osseous metastatic disease to the hands and feet. Variables recorded included primary cancer histology, age at diagnosis of primary and acrometastases, location of acrometastases, burden of disease, treatment, and age at death. Kaplan-Meier curves were used to estimate survival, and log-rank tests were used for comparison. The median age at acrometastases diagnosis was 61.5 years, and the most common associated primary cancer was lung carcinoma (n=9). Sixteen patients had acrometastases to the foot, and 12 had acrometastases to the hand. In the foot, most acrometastases were located in the tarsal bones (n=11), followed by the metatarsals (n=4) and the phalanges (n=3). In the hand, most were in the metacarpals and the phalanges (n=6 each), and 2 were in the carpals. The most common treatment was resection or curettage only (n=7). The median duration of survival after acrometastases diagnosis was 9.7 months. Despite advances in cancer treatment and shifting patterns of metastatic disease, there has been little change in the distribution of primary malignancies responsible for acrometastases, with lung carcinoma remaining the most common. Ablative procedures are rarely required, with limb-salvage interventions predominating. [Orthopedics. 2019; 42(2):e197-e201.]., (Copyright 2018, SLACK Incorporated.)

Background: Patients with recurrent or refractory osteosarcoma have a poor prognosis with less than 30% surviving two years. Eribulin is a synthetic analog of halichondrin B, has a novel mechanism of action when compared with other microtubule inhibitors, and may have antitumor activity in osteosarcoma., Methods: A prospective study was designed to assess the disease control success at four months and objective response rates in patients with recurrent or refractory osteosarcoma treated with eribulin. Eligible patients were between 12 and 50 years of age, had measurable tumor, and met standard organ function requirements. Patients were given eribulin 1.4 mg/m 2 /dose on days 1 and 8 of each 3-week cycle for up to 24 months if there was no progressive disease. Response to therapy was assessed using RECIST 1.1 criteria after cycles 2 and 5 and every fourth cycle thereafter., Results: Nineteen patients enrolled on the AOST1322 study. The median age of enrollment was 16 years (range, 12-25 years). Twelve patients were male and seven female. Eribulin was well tolerated, with neutropenia identified as the most common toxicity. The median progression-free survival was 38 days and no patients reached the four-month time point without progression. No objective responses were seen in any patient., Conclusion: This study rapidly assessed the clinical activity of a novel agent in this patient population. Eribulin was well tolerated, but there were no patients who demonstrated objective response, and all patients had progression prior to four months., (© 2018 Wiley Periodicals, Inc.)

Background: Surgical decision-making can be challenging when treating patients with osseous metastases. Numerous factors, including expected duration of survival, must be considered to ensure optimal operative stabilization of the affected bone. However, life expectancy of patients with metastatic carcinoma is often difficult to estimate. The goal of our study was to assess the associations of various clinical and demographic factors with survival time after intramedullary nail fixation of impending or completed pathologic femur fractures., Methods: One hundred thirty-eight consecutive patients treated with intramedullary nail fixation for impending or completed pathologic femur fractures between 2005 and 2017 were included in this study. Factors related to patient survival were assessed with Cox multivariate survival analysis. For all analyses, p < 0.05 was considered significant., Results: The median overall postoperative survival time was 8.4 months. Lower hemoglobin concentration (p = 0.001), lower albumin concentration (p = 0.002), and having a group 2 primary cancer (p = 0.001) were associated with shorter survival on multivariate analysis. When considering the subgroup of 88 prophylactically stabilized patients, lower hemoglobin concentration (p = 0.005), lower albumin concentration (p = 0.015), and having a group 2 primary cancer (p = 0.037) were predictive of shorter survival., Conclusion: Several factors are associated with shorter survival after intramedullary nail fixation of pathologic femur fractures. These factors should be considered by orthopedic surgeons when educating patients and determining appropriate treatment., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Background and Objectives: A lack of consensus exists on the prognosis of extraskeletal Ewing sarcoma (EES) relative to its skeletal (ES) counterpart in adults. This study sought to characterize outcome differences between the two diagnoses., Methods: From 2004 to 2014, the NCDB identified 2,660 Ewing Sarcoma patients. Cox proportional hazards regression analysis was used to identify risk factors for overall survival (OS)., Results: EES patients were older, more likely to be female, and have smaller tumors. Among patients with ES, 4.0% received no treatment, 2.5% received local therapy only (surgery and/or radiation), 16.8% received chemotherapy only, while 52.2% received combination therapy (local and chemotherapy), and 17.0% recieived triple therapy (surgery, radiation and chemotherapy). Among patients with EES, 4.3% recived no treatment, 5.6% received local therapy only, 15.6% received chemotherapy only, while 47.0% received combination therapy, and 21.6% received triple therapy. No difference in OS was observed between the two groups (P = 0.816). Factors independently associated with OS for ES included age (HR = 1.26, P = 0.01), Charlson-Deyo Score (CDS) ≥2 (HR = 3.66, P < 0.001), combination therapy (HR = 0.39, P < 0.001) and triple therapy (HR = 0.34, P < 0.001). For EES, factors for OS were age (HR = 1.52, P < 0.001), CDS ≥2 (HR = 1.90, P = 0.02), combination therapy (HR = 0.44, P < 0.001), triple therapy (HR = 0.34, P < 0.001) and PNET histology (HR = 1.33, P = 0.02)., Conclusions: Demographic, histological, and treatment characteristics differ between adult patients diagnosed with ES and ESS. However, survival and independent predictors of survival are consistent between the two diagnoses., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Chordomas are low-grade malignant tumors that are locally aggressive and have the potential to metastasize. They most often occur in the sacrococcygeal and spheno-occipital portions of the vertebral column. Morphologically similar tumors have been found outside the axial skeleton and are referred to as extra-axial chordomas. Several case reports have described the radiologic, microscopic, and immunologic profiles of these tumors and their similarities to axial chordomas. The authors report a 24-year-old man who presented with a mass in his left hand and underwent surgical excision. Specimens stained positive for pancytokeratin, S100, and brachyury. Brachyury is a protein that is present during embryogenesis and is expressed by chordomas. This is the first report of an extra-axial chordoma within the interosseous muscle compartment of the hand in a young patient., (Copyright © 2017 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.)

Case: We report the case of a 50-year-old woman with oncogenic osteomalacia secondary to a metastatic phosphaturic mesenchymal tumor (PMT) that presented, to our knowledge, with the first reported lesion in the talus., Conclusion: Oncogenic osteomalacia is a rare condition with a unique serum biochemical profile that requires a high index of suspicion for diagnosis. A PMT is a rare neoplasm that can lead to oncogenic osteomalacia through secretion of fibroblast growth factor 23. Symptoms can be debilitating, and diagnostic delays are extremely common. This case report emphasizes the importance of comprehensive anatomic assessment and the need for fastidious postoperative monitoring.

Reconstruction options in children after bone tumor resection are as varied as they are challenging. Advances in biologic and endoprosthetic design have led to many choices, all of which must be considered in the context of prognosis, treatment limitations, and patient/family expectations. The current experience and results of limb-sparing surgery following bone sarcoma resection in growing children are discussed, including allograft, autograft, and metallic prostheses alone and in combination, especially as they pertain to the knee. In some cases, the ablative options of amputation and rotationplasty must be seen as equal and, at times, superior choices to limb salvage.

The NCCN Guidelines for Bone Cancer provide interdisciplinary recommendations for treating chordoma, chondrosarcoma, giant cell tumor of bone, Ewing sarcoma, and osteosarcoma. These NCCN Guidelines Insights summarize the NCCN Bone Cancer Panel's guideline recommendations for treating Ewing sarcoma. The data underlying these treatment recommendations are also discussed., (Copyright © 2017 by the National Comprehensive Cancer Network.)

Background: Even though virtually all patients with Ewing sarcoma achieve a radiographic complete response, up to 30% of patients who present with localized disease and up to 90% of those who present with metastases experience a metastatic disease recurrence, highlighting the inability to identify patients with residual disease at the end of therapy. Up to 95% of Ewing sarcomas carry a driving EWS-ETS translocation that has an intronic breakpoint that is specific to each tumor, and the authors developed a system to quantitatively detect the specific breakpoint DNA fragment in patient plasma., Methods: The authors used a long-range multiplex polymerase chain reaction (PCR) technique to identify tumor-specific EWS-ETS breakpoints in Ewing sarcoma cell lines, patient-derived xenografts, and patient tumors, and this sequence was used to design tumor-specific primer sets to detect plasma tumor DNA (ptDNA) by droplet digital PCR in xenograft-bearing mice and patients., Results: Tumor-specific breakpoint DNA fragments were detected in the plasma of xenograft-bearing mice, and the signal correlated with tumor burden during primary tumor growth, after surgical resection, and at the time of metastatic disease recurrence. Furthermore, the authors were able to detect the specific breakpoint in plasma DNA obtained from 3 patients with Ewing sarcoma and in 2 patients the authors were able to detect ptDNA when there was radiographically undetectable disease present., Conclusions: The use of droplet digital PCR to detect tumor-specific EWS-ETS fusion gene breakpoint ptDNA fragments can be developed into a highly personalized biomarker of disease recurrence that can be optimized in animal studies for ultimate use in patients. Cancer 2016;122:3015-3023. © 2016 American Cancer Society., Competing Interests: The authors have declared that no conflict of interest exists., (© 2016 American Cancer Society.)

Primary musculoskeletal cancer and metastatic disease to bone in pregnant patients presents major treatment challenges. Although uncommon, musculoskeletal malignancies in pregnant women have been reported. When diagnosing and treating these patients, the mother's health must be managed appropriately while ensuring that fetal development is not deleteriously affected. Extensive radiographic imaging and more advanced techniques are often necessary to fully characterize the extent of disease. When possible, magnetic resonance imaging should be used instead of computed tomography to limit exposure of the conceptus to radiation. If treatment is needed, therapeutic radiation, chemotherapy, and surgery should be considered. Surgical resection is the foundation of treatment of early-stage primary bone tumors and soft-tissue sarcomas during pregnancy. With surgery, anesthesia and thromboprophylaxis are important considerations. If chemotherapy is required, administration should be avoided in the first trimester to limit harm to the fetus. Therapeutic radiation should similarly be avoided during the first trimester and often can be postponed until after delivery., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

Background: Complete surgical resection of primary tumors is critical for long-term control of high-grade osteosarcoma. Uniform assessment of the extent of surgical resection is important in clinical trials, though the accuracy of this reporting has been poorly studied., Methods: We conducted a retrospective cohort study of patients 5-40 years of age with newly diagnosed high-grade resectable osteosarcoma treated as part of the AOST0331 clinical trial at Children's Oncology Group institutions. The extent of surgical resection of the primary tumor was graded as wide or radical by the treating institution. Central assessment of the extent of resection by two orthopedic oncologists was compared with institutional assessment by reviewing pathology and operative reports., Results: We included 956 patients who had data available for central review. The extent of resection reported by treating institutions was 536/956 (56%) radical and 420/956 (44%) wide. The extent of resection assessed by central review was 162/956 (17%) radical and 794/956 (83%) wide. The overall discordance rate for the cohort was 43%., Conclusions: Institutional reports of radical resection in high-grade osteosarcoma significantly over-estimate the proportion of patients undergoing radical resection. This highlights the need for centralized review and improved accuracy of reporting of the extent of resection. J. Surg. Oncol. 2016;113:351-354. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Background: Lower extremity reconstruction after resection of long bone tumors in children is challenging because of the unique functional demands and growth potential of the lower extremity. The use of a free fibula flap inside a massive bone allograft provides a reliable reconstructive option. The authors evaluate the surgical and functional outcomes of using this technique., Methods: This is a retrospective review of 12 consecutive patients who underwent reconstruction of segmental femur or tibia defects using a free fibula flap inside a massive bone allograft between 2003 and 2011. Complications and functional outcomes are reported., Results: Twelve patients with a mean age of 15.8 years (range, 3 to 49 years) were included in the study. Eight femur defects and four tibia defects were reconstructed. The mean follow-up time was 41.4 months. Two constructs were removed because of infection, three patients required bone grafting for nonunion, one patient required an additional operation to excise a skin paddle, and one patient experienced a lower extremity deep vein thrombosis. The mean time to achieve full weight bearing was 14.3 months., Conclusions: The use of a free fibula flap inside a massive bone allograft after bone tumor resection provides an option for lower extremity reconstruction. The allograft component increases the initial strength of the reconstruction, whereas the vascularized fibula component is thought to increase the biologic potential for osteosynthesis and ultimately provide a potentially lifelong durable reconstruction. Patients who achieve oncologic control are likely to enjoy a highly functional long-term outcome., Clinical Question/level of Evidence: Therapeutic, IV.

Purpose: EURAMOS-1, an international randomized controlled trial, investigated maintenance therapy with pegylated interferon alfa-2b (IFN-α-2b) in patients whose osteosarcoma showed good histologic response (good response) to induction chemotherapy., Patients and Methods: At diagnosis, patients age ≤ 40 years with resectable high-grade osteosarcoma were registered. Eligibility after surgery for good response random assignment included ≥ two cycles of preoperative MAP (methotrexate, doxorubicin, and cisplatin), macroscopically complete surgery of primary tumor, < 10% viable tumor, and no disease progression. These patients were randomly assigned to four additional cycles MAP with or without IFN-α-2b (0.5 to 1.0 μg/kg per week subcutaneously, after chemotherapy until 2 years postregistration). Outcome measures were event-free survival (EFS; primary) and overall survival and toxicity (secondary)., Results: Good response was reported in 1,041 of 2,260 registered patients; 716 consented to random assignment (MAP, n = 359; MAP plus IFN-α-2b, n = 357), with baseline characteristics balanced by arm. A total of 271 of 357 started IFN-α-2b; 105 stopped early, and 38 continued to receive treatment at data freeze. Refusal and toxicity were the main reasons for never starting IFN-α-2b and for stopping prematurely, respectively. Median IFN-α-2b duration, if started, was 67 weeks. A total of 133 of 268 patients who started IFN-α-2b and provided toxicity information reported grade ≥ 3 toxicity during IFN-α-2b treatment. With median follow-up of 44 months, 3-year EFS for all 716 randomly assigned patients was 76% (95% CI, 72% to 79%); 174 EFS events were reported (MAP, n = 93; MAP plus IFN-α-2b, n = 81). Hazard ratio was 0.83 (95% CI, 0.61 to 1.12; P = .214) from an adjusted Cox model., Conclusion: At the preplanned analysis time, MAP plus IFN-α-2b was not statistically different from MAP alone. A considerable proportion of patients never started IFN-α-2b or stopped prematurely. Long-term follow-up for events and survival continues.

At some point in their careers, many orthopaedic surgeons will have to navigate the legal system as it pertains to medical malpractice. An orthopaedic surgeon will find it helpful to review information on the basic legal elements of medical malpractice law along with suggestions on how he or she can assist the legal defense team if a lawsuit is filed. Surgeons who face litigation within the context of managing patients with musculoskeletal tumors should be aware of the common pitfalls in managing these patients. Knowledge of complementary strategies can provide good patient care and reduce legal risks when caring for patients with musculoskeletal neoplasms.

Primary bone cancers are extremely rare neoplasms, accounting for fewer than 0.2% of all cancers. The evaluation and treatment of patients with bone cancers requires a multidisciplinary team of physicians, including musculoskeletal, medical, and radiation oncologists, and surgeons and radiologists with demonstrated expertise in the management of these tumors. Long-term surveillance and follow-up are necessary for the management of treatment late effects related to surgery, radiation therapy, and chemotherapy. These guidelines discuss the management of chordoma, giant cell tumor of the bone, and osteosarcoma.

Although primary malignancies of bone are rare, thousands of benign bone tumors are diagnosed annually. It is important to be able to distinguish benign lesions from malignant lesions and differentiate those lesions that can be watched versus lesions that require further treatment and referral to an orthopaedic oncologist. Learning to distinguish these entities and their appropriate treatment or triage will positively affect the patient and the surgeon's practice.

Background: The pelvis is an infrequent site of osteosarcoma and treatment requires surgery plus systemic chemotherapy. Poor survival has been reported, but has not been confirmed previously by the Children's Oncology Group (COG). In addition, survival of patients with pelvic osteosarcomas has not been compared directly with that of patients with nonpelvic disease treated on the same clinical trials., Questions/purposes: First, we assessed the event-free (EFS) and overall survival (OS) of patients with pelvic osteosarcoma treated on COG clinical trials. We then asked whether patient survival compared with that of patients treated on the same clinical trials with nonpelvic disease. Finally, we asked whether patients with metastatic disease at initial diagnosis had worse survival., Methods: We retrospectively reviewed data from 1054 patients with osteosarcoma treated in four studies between 1993 and 2005. Twenty-six of the 1054 patients (2.5%) had a primary tumor of the pelvis. At diagnosis, nine patients had metastatic disease. The minimum followup was 2 months (mean, 34 months; range, 2-102 months)., Results: Two of the nine patients with metastatic disease at diagnosis and five of the 17 with localized disease were alive at last contact. Estimates of the 5-year EFS for localized versus metastatic disease of the pelvis were 22% versus 23%. OS for patients with localized versus metastatic disease was 47% versus 22%. Patients with osteosarcoma in all other locations had a 5-year EFS of 57% and OS of 69%., Conclusions: Our analysis confirms poor survival for patients with pelvic osteosarcoma. Survival with metastatic disease in the absence of a pelvic primary tumor is similar to that for localized or metastatic pelvic osteosarcoma. Improved surgical or medical therapy is needed, and patients with pelvic osteosarcoma may warrant alternate or experimental therapy.

Background: Pathologic proximal femur fractures result in substantial morbidity for patients with skeletal metastases. Surgical treatment is widely regarded as effective; however, failure rates associated with the most commonly used operative treatments are not well defined., Questions/purposes: We therefore compared surgical treatment failure rates among intramedullary nailing, endoprosthetic reconstruction, and open reduction-internal fixation when applied to impending or displaced pathologic proximal femur fractures., Patients and Methods: We retrospectively compared the clinical course of 298 patients who underwent intramedullary nailing (n = 82), endoprosthetic reconstruction (n = 197), or open reduction-internal fixation (n = 19) from 1993 to 2008. Primary outcome was treatment failure, which was defined as reoperation for any reason. Treatment groups were compared for differences in demographic and clinical parameters., Results: The number of treatment failures in the endoprosthetic reconstruction group (3.1%) was significantly lower than in the intramedullary nailing (6.1%) and open reduction-internal fixation (42.1%) groups. The number of revisions requiring implant exchange also was significantly lower for endoprosthetic reconstruction (0.5%), compared with intramedullary nailing (6.1%) and open reduction-internal fixation (42.1%)., Conclusions: Endoprosthetic reconstruction is associated with fewer treatment failures and greater implant durability. Prospective studies are needed to determine the impact of operative strategy on function and quality of life., Level of Evidence: Level III, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

There is considerable overlap in the clinical and imaging presentation of general orthopaedic conditions and musculoskeletal neoplasms. At centers that treat orthopaedic oncologic conditions, it is not uncommon to see patients with spine and extremity tumors previously treated for presumed general orthopaedic ailments. It is important for orthopaedic surgeons to understand how to interpret commonly ordered radiographic studies (radiographs, MRIs, and CT scans) as they relate to bone and soft-tissue tumors, to be familiar with the imaging appearance of common musculoskeletal lesions in the extremities and spine, and to understand what imaging findings should trigger a referral to an orthopaedic oncologist.

Background: This study evaluated the safety and feasibility of the addition of pamidronate to chemotherapy for treatment of osteosarcoma., Methods: The authors treated 40 patients with osteosarcoma with cisplatin, doxorubicin, and methotrexate with the addition of pamidronate 2 mg/kg/dose (max dose 90 mg) monthly for 12 doses. Survival, event-free survival (EFS), and durability of orthopedic reconstruction were evaluated., Results: For patients with localized disease, event-free survival (EFS) at 5 years was 72% and overall survival 93%. For patients with metastatic disease, EFS at 5 years was 45% and overall survival 64%. Toxicity was similar to patients treated with chemotherapy alone. Thirteen of 14 uncemented implants demonstrated successful osteointegration. Among allograft reconstructions, there were 2 graft failures, 4 delayed unions, and 6 successful grafts. Overall, 5 of 33 reconstructions failed. There were no stress fractures or growth disturbances., Conclusions: Pamidronate can be safely incorporated with chemotherapy for the treatment of osteosarcoma. It does not impair the efficacy of chemotherapy. Pamidronate may improve the durability of limb reconstruction., (Copyright © 2010 American Cancer Society.)

Treatment of pelvic bone sarcomas remains one of the most challenging areas of orthopedic oncology for all members of the disease management team. Although considerable advances have been made in all aspects of sarcoma treatment, the prognosis for patients with primary sarcomas of the pelvis continues to be guarded, and therefore, much controversy exists regarding optimal surgical management, radiation therapy, and systemic therapy. This article summarizes the current treatment paradigms for the 3 most common bone sarcomas (osteosarcoma, Ewing's sarcoma, and chondrosarcoma), highlighting the unresolved issues in their management as they pertain to the pelvis.

Purpose of Review: Osteogenic sarcoma, also known as osteosarcoma, is a primary malignancy of the bone in which spindle cells produce osteoid. Although rare in the general population, these tumors are the most common primary malignancies of bone and the fifth most common primary malignancy of adolescence. This paper aims to provide a general overview on the presentation, radiographic evaluation, and treatment of osteosarcoma as it presents in the pediatric patient population. Particular focus is placed on the importance of a comprehensive team of physicians, including the pediatric oncologist and orthopedic surgeon, to ensure prompt diagnosis and treatment., Recent Findings: Survival for osteosarcoma tumor has changed significantly due to advanced chemotherapeutic regimens; current data place 5-year survival rates at 60% or greater. In addition, limb-sparing surgery has largely supplanted amputation, though the effect of this change on functional outcomes is controversial. Recent studies have shown greatly improved long-term survival data using current therapeutic regimens at tertiary referral centers., Summary: Advancement in chemotherapeutic regimens has allowed improved survival and limb-sparing surgery in the treatment of osteosarcoma.

Unlabelled: Bone ingrowth promises more durable biologic fixation of megaprostheses. The relative performance of different types of fixation is unknown. We compared the fixation of two forms of biologically fixed femoral components: an intramedullary uncemented press-fit stem (UCS; Group 1, 50 patients) and a Compress((R)) uncemented fixation (CPS; Group 2, 41 patients). In Group 1, the overall Kaplan-Meier prosthetic survival rates were 85% at 5 and 71% at 10 years. Most failures were long-term developments. Aseptic loosening was the primary cause of failure. Stem diameters less than 13.5 mm and a diaphyseal/stem coefficient greater than 2.5 mm were associated with decreased prosthetic survival. In Group 2, the overall rate of CPS survival was 88% at 5 years. Failure of femoral fixation or fracture during the first year was the main reason for revision. Five-year survival rates were similar between the groups and we observed no difference in the functional success of the implants. We found no failures after 1-year followup in Group 2 (CPS). Any difference in prosthetic survival can only be proven by longer-term study or a randomized trial., Level of Evidence: Level III, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Unlabelled: Periprosthetic fractures after massive endoprosthetic reconstructions pose a reconstructive challenge and jeopardize limb preservation. Compressive osseointegration technology offers the promise of relative ease of prosthetic revision, since fixation is achieved by means of a short intramedullary device. We retrospectively reviewed the charts of 221 patients who had Compress((R)) devices implanted in two centers between December, 1996 and December, 2008. The mean followup was 50 months (range, 1-123 months). Six patients (2.7%) sustained periprosthetic fractures and eight (3.6%) had nonperiprosthetic ipsilateral limb fractures occurring from 4 to 79 months postoperatively. All periprosthetic fractures occurred in patients with distal femoral implants (6/154, 3.9%). Surgery was performed in all six patients with periprosthetic femur fractures and for one with a nonperiprosthetic patellar fracture. The osseointegrated interface was radiographically stable in all 14 cases. All six patients with periprosthetic fracture underwent limb salvage procedures. Five patients had prosthetic revision; one patient who had internal fixation of the fracture ultimately underwent amputation for persistent infection. Periprosthetic fractures involving Compress((R)) fixation occur infrequently and most can be treated successfully with further surgery. When implant revision is needed, the bone preserved by virtue of using a shorter intramedullary Compress((R)) device as compared to conventional stems, allows for less complex surgery, making limb preservation more likely., Level of Evidence: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Background: Limb salvage following resection of a tumor in the proximal part of the humerus poses many challenges. Reconstructive options are limited because of the loss of periarticular soft-tissue stabilizers of the glenohumeral joint in addition to the loss of bone and articular cartilage. The purpose of this study was to evaluate the functional outcome and survival of the reconstruction following use of a humeral allograft-prosthesis composite for limb salvage., Methods: An allograft-prosthesis composite was used to reconstruct a proximal humeral defect following tumor resection in thirty-six consecutive patients at one institution over a sixteen-year period. The reconstruction was performed at the time of a primary tumor resection in thirty cases, after a failure of a reconstruction following a previous tumor resection in five patients, and following excision of a local recurrence in one patient. The mean duration of follow-up of the living patients was five years. Glenohumeral stability, function, implant survival, fracture rate, and union rate following the reconstructions were measured. Functional outcome and implant survival were analyzed on the basis of the amount of deltoid resection, whether the glenohumeral resection had been extra-articular or intra-articular, and the length of the humerus that had been resected., Results: One patient sustained a glenohumeral dislocation. Deltoid resection (partial or complete) resulted in a reduced postoperative range of motion in flexion and abduction but had no effect on the mean Musculoskeletal Tumor Society score. Extra-articular resections were associated with lower Musculoskeletal Tumor Society scores. All patients had either mild or no pain and normal hand function at the time of final follow-up. The overall estimated rate of survival of the construct, with revision as the end point, was 88% at ten years. There were three failures due to progressive prosthetic loosening that necessitated removal of the construct. Four patients required an additional bone-grafting procedure to treat a delayed union of the osteosynthesis site., Conclusions: An allograft-prosthesis composite used for limb salvage following tumor resection in the proximal part of the humerus is a durable construct associated with an acceptable complication rate. Deltoid preservation and intra-articular resection are associated with a greater range of shoulder motion and a superior functional outcome, respectively.

Unlabelled: We propose a surgical technique for structural allograft reconstitution of the diaphysis of long bones, maximizing surface contact between host and allograft bone. This method, analogous to a telescope, overlaps the graft and host bone, theoretically increasing bone surface contact substantially. We report the outcome of 22 telescoped allograft junction sites in 19 patients who lacked sufficient host bone to accommodate a regular-length stemmed implant. This joint-sparing reconstruction preserved 15 of 16 adjacent joints at risk for replacement. Five patients needed additional surgery, but none for nonunion. The diaphyseal length could be reconstructed enough so that a short prosthesis (less than the critical 40% of total bone length) could be used. This biologic method to reconstruct major segments of the diaphysis is best suited for patients with quantitatively or qualitatively deficient residual bone stock after tumor resection or prosthetic revision. We believe it is an excellent technique for revision knee megaprostheses when there is a short remnant of proximal femur., Level of Evidence: Level IV, therapeutic study.

Tumors involving the joint or having symptoms in the joint are rare. Both joint-related tumors and sports-related injuries can affect young, active patients, and their symptoms often overlap. Sports medicine specialists rarely encounter synovial conditions, so expertise in this area is difficult to establish. Orthopaedic oncologists often see only patients with an advanced condition. The clinical presentation of a soft-tissue sarcoma may be similar to that of a common lesion such as a synovial cyst. Some benign or malignant bone tumors cause referred pain to distant joints, possibly leading to a delay in diagnosis or inappropriate initial surgery. For example, a hip or proximal femoral bone tumor commonly causes isolated knee pain. Conversely, because the symptoms of some sports-related conditions or pseudotumors (such as a rectus femoris tear, fascial herniation, myositis ossificans, an avulsion injury, an avulsive cortical irregularity, femoral diaphyseal periostitis, or pseudotumor deltoideus) are similar to symptoms of a sarcoma, overtreatment is possible. A sports medicine physician should be familiar with these conditions to facilitate accurate and expedient diagnosis with appropriate treatment.

Bone scans are widely utilized for detection of metastases from bone sarcomas. Technetium methylene diphosphonate scan ([(99m)Tc]MDP) is one of the most popular radiotracers used for that purpose. Lymphatic spread of bone sarcomas is unusual and often difficult to diagnose. Unfortunately, bone scans are not as sensitive in demonstrating lymphatic spread of sarcomas as they are at demonstrating hematogenous spread. A bone scan will often fail to demonstrate lymph nodes metastases until there is mineralization at the affected node. In this report, we highlight an interesting case of a patient with secondary osteogenic sarcoma (OS) from Paget's disease in the distal femur with non-ossified inguinal nodal metastasis diagnosed with [(99m)Tc]MDP. Lymph node involvement was not appreciated on plain radiographs or computed tomography (CT).

Unlabelled: While low-grade juxtacortical and low-grade intramedullary osteogenic sarcomas are histologically indistinguishable, they have been studied as separate entities. We retrospectively reviewed the clinical, radiographic, histologic features and treatment of 59 patients treated surgically to compare the rate of local recurrence, grade progression, and survival between low-grade intramedullary and low-grade juxtacortical osteogenic sarcoma. Forty-five (76%) patients were treated for low-grade juxtacortical osteogenic sarcoma and 14 (24%) were treated for low-grade intramedullary osteogenic sarcoma. Local recurrence rates of 7% were similar for both groups studied. The rate of distant metastases was also similar for both groups. . The rate of dedifferentiation for the entire group was 29%. Dedifferentiated lesions were treated with adjuvant chemotherapy in 16 of 17 cases. Recurrence preceded dedifferentiation in four cases. Five-year survival was over 90% in both groups. Low-grade intramedullary and low-grade juxtacortical osteogenic sarcoma were clinically indistinguishable with identical rates of local recurrence, distant metastases, dedifferentiation, and survival., Level of Evidence: Level III, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.

The prevalence of intravascular extension of bone sarcomas of the pelvis is unknown. We report a case of intravascular extension of an osteogenic sarcoma of the pelvis into the vena cava, correlating the pre-operative imaging findings with the intra-operative findings and specimen analysis. A brief review of the literature is provided.

Quality of life measures have neglected to include a critical self-assessment component in pediatric sarcoma patients. Our report shows how children rate their own quality of life and how that varies over time after surgery. Using the Pediatric Outcomes Data Collection Instrument, quality of life data was prospectively collected and combined with a retrospective review of clinical parameters on 43 children with primary bone sarcoma, with an average followup of 3 years. Children reported good yet variable scores in five of the six domains. Lower scores were noted in the Sports/Physical Functioning domain, particularly in the first 12 months after surgery, with improvement seen up to 24 months after surgery. Tumor specific factors such as size larger than 8 cm and lower extremity location were negative predictors for Sports/Physical Functioning. The only demographic factor that predicted perceived quality of life scores was gender, with girls reporting lower scores in Sports/Physical Functioning, Pain/Comfort, and Global Functioning domains. The Pediatric Outcomes Data Collection Instrument gives discriminatory detailed textured evaluation of the outcome of children treated for skeletal sarcoma. Further development of quality of life measures is needed to allow its use in treatment selection.

Advocates of scapular replacement suggest sparing the deltoid should be a prerequisite for use of a scapular prosthesis. We evaluated the importance of a functioning deltoid in scapular replacement for malignant tumors. We retrospectively reviewed 19 patients who received scapular prostheses after resection of a malignant tumor. The median followup was 18 months (range, 12-124 months). The axillary nerve was resected in eight patients and spared in 11 patients. The average International Society of Limb Salvage score was 25 of 30 points (range, 21-27 points). The average scores for patients with and without axillary nerve resection were 24 points (range, 21-25 points) and 26 points (range, 23-27 points), respectively. There was a difference between the two groups in hand positioning, overall function, and pain. However, there were no differences in emotional acceptance, lifting ability, or hand dexterity. We favor using a scapular prosthesis if there is a functioning deltoid; however resection of the deltoid should not be considered an absolute contraindication for scapular replacement.