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Purpose: Metastatic disease of the pelvis is frequently associated with severe pain and impaired ambulatory function. Depending on the patient's characteristics, primary tumor, and metastatic pelvic disease, the treatment choice may be varied. This study aims to report on the current management options of metastatic pelvic disease., Methods: We comprehensively researched multiple databases and evaluated essential studies about current concepts of managing a metastatic bone disease of the pelvis, focusing on specific indications as well as on the result of treatment., Results: Pelvic metastases not in the periacetabular region can be managed with modification of weight-bearing, analgesics, bisphosphonates, chemotherapy and/or radiotherapy. Minimally invasive approaches include radiofrequency ablation, cryoablation, embolization, percutaneous osteoplasty, and percutaneous screw placement. Pathological or impending periacetabular fracture, excessive periacetabular bone defect, radioresistant tumor, and persistent debilitating pain despite non-surgical treatment and/or minimally invasive procedures can be managed with different surgical techniques. Overall, treatment can be divided into nonoperative, minimally invasive, and operative based on specific indications, the expectations of the patient and the lesion., Conclusion: Different treatment modalities exist to manage metastatic pelvic bone disease. Decision-making for the most appropriate treatment should be made with a multidisciplinary approach based on a case-by-case basis., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)

Chondrosarcoma is the second most common surgically treated primary bone sarcoma. Despite a large number of scientific papers in the literature, there is still significant controversy about diagnostics, treatment of the primary tumour, subtypes, and complications. Therefore, consensus on its day-to-day treatment decisions is needed. In January 2024, the Birmingham Orthopaedic Oncology Meeting (BOOM) attempted to gain global consensus from 300 delegates from over 50 countries. The meeting focused on these critical areas and aimed to generate consensus statements based on evidence amalgamation and expert opinion from diverse geographical regions. In parallel, periprosthetic joint infection (PJI) in oncological reconstructions poses unique challenges due to factors such as adjuvant treatments, large exposures, and the complexity of surgery. The meeting debated two-stage revisions, antibiotic prophylaxis, managing acute PJI in patients undergoing chemotherapy, and defining the best strategies for wound management and allograft reconstruction. The objectives of the meeting extended beyond resolving immediate controversies. It sought to foster global collaboration among specialists attending the meeting, and to encourage future research projects to address unsolved dilemmas. By highlighting areas of disagreement and promoting collaborative research endeavours, this initiative aims to enhance treatment standards and potentially improve outcomes for patients globally. This paper sets out some of the controversies and questions that were debated in the meeting., Competing Interests: A. Puri is a member of the editorial board of The Bone & Joint Journal. P. Ruggieri reports consulting fees from Exactech and Stryker, unrelated to this study. M. T. Houdek reports consulting fees from Link Orthopedics, unrelated to this study. E. Botello reports consulting fees, payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events, and support for attending meetings and/or travel from Zimmer Biomet, all of which are unrelated to this study. G. V. Morris reports consulting fees, payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events, and support for attending meetings and/or travel from Implantcast, all of which are unrelated to this study., (© 2024 Jeys et al.)

Giant cell tumor of bone (GCTB) is characterized by uncertain biological behavior due to its local aggressiveness and metastasizing potential. In this study, we conducted a meta-analysis of the contemporary literature to evaluate all management strategies for GCTB metastases. A combination of the terms "lung metastases", "giant cell tumor", "bone", "treatment", and "oncologic outcomes" returned 133 patients meeting our inclusion criteria: 64 males and 69 females, with a median age of 28 years (7-63), at the onset of primary GCTB. Lung metastases typically occur at a mean interval of 26 months (range: 0-143 months) after treatment of the primary site, commonly presenting as multiple and bilateral lesions. Various treatment approaches, including surgery, chemotherapy, radiotherapy, and drug administration, were employed, while 35 patients underwent routine monitoring only. Upon a mean follow-up of about 7 years (range: 1-32 years), 90% of patients were found to be alive, while 10% had died. Death occurred in 25% of patients who had chemotherapy, whereas 96% of those not treated or treated with Denosumab alone were alive at a mean follow-up of 6 years (range: 1-19 years). Given the typically favorable prognosis of lung metastases in patients with GCTB, additional interventions beyond a histological diagnosis confirmation may not be needed. Denosumab, by reducing the progression of the disease, can play a pivotal role in averting or delaying lung failure.

Primary vascular tumors of bone are a heterogeneous group of neoplasms, ranging from benign hemangiomas to frankly malignant epithelioid hemangioendotheliomas and angiosarcomas. Over the years, their classification has been a matter of discussion, due to morphologic similarities and uncertainty regarding biologic behavior. Over the past decade, with the development of next-generation sequencing, there has been a significant improvement in the molecular characterization of these lesions. The integration of their morphologic, immunohistochemical and molecular features has led to a better stratification, with important prognostic and therapeutic implications. Nevertheless, primary vascular bone tumors still represent a challenge for medical oncologists. Given their rarity and heterogeneity, in the last few years, there has been no significant progress in medical treatment options, so further research is needed. Here we present a review of the current knowledge regarding primary vascular tumors of the bone, correlating clinicopathologic features with tumor behavior and therapeutic approaches., Competing Interests: Declaration of Competing Interest PR reports personal fees as Consultant for Exactech and Stryker. AB reports serving on advisory boards for Eli Lilly, Roche, GSK, Eisai, Pharmamar, Boehringer Ingelheim, Deciphera; personal fees or honoraria for educational events by GSK and Pharmamar; travel grants by Gentili and Pharmamar. All the authors, including PR and AB, declare no competing interests with respect to this research., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Background: Surgical site infection (SSI) after segmental endoprosthetic reconstruction in patients treated for oncologic conditions remains both a devastating and a common complication. The goal of the present study was to identify variables associated with the success or failure of treatment of early SSI following the treatment of a primary bone tumor with use of a segmental endoprosthesis., Methods: The present study used the Prophylactic Antibiotic Regimens in Tumor Surgery (PARITY) data set to identify patients who had been diagnosed with an SSI after undergoing endoprosthetic reconstruction of a lower extremity primary bone tumor. The primary outcome of interest in the present study was a dichotomous variable: the success or failure of infection treatment. We defined failure as the inability to eradicate the infection, which we considered as an outcome of amputation or limb retention with chronic antibiotic suppression (>90 days or ongoing therapy at the conclusion of the study). Multivariable models were created with covariates of interest for each of the following: surgery characteristics, cancer treatment-related characteristics, and tumor characteristics. Multivariable testing included variables selected on the basis of known associations with infection or results of the univariable tests., Results: Of the 96 patients who were diagnosed with an SSI, 27 (28%) had successful eradication of the infection and 69 had treatment failure. Baseline and index procedure variables showing significant association with SSI treatment outcome were moderate/large amounts of fascial excision ≥1 cm2) (OR, 10.21 [95% CI, 2.65 to 46.21]; p = 0.001), use of local muscle/skin graft (OR,11.88 [95% CI, 1.83 to 245.83]; p = 0.031), and use of a deep Hemovac (OR, 0.24 [95% CI, 0.05 to 0.85]; p = 0.041). In the final multivariable model, excision of fascia during primary tumor resection was the only variable with a significant association with treatment outcome (OR, 10.21 [95% CI, 2.65 to 46.21]; p = 0.018)., Conclusions: The results of this secondary analysis of the PARITY trial data provide further insight into the patient-, disease-, and treatment-specific associations with SSI treatment outcomes, which may help to inform decision-making and management of SSI in patients who have undergone segmental bone reconstruction of the femur or tibia for oncologic indications., Level of Evidence: Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJS/H576)., (Copyright © 2023 by The Journal of Bone and Joint Surgery, Incorporated.)

Purpose: Modular megaprostheses have widely replaced allografts, as a reconstructive option; however, failures of these devices remain high. Aim of this study was to analyze outcomes, survival of the implants, incidence and types of complications with Mutars modular endoprostheses at long term., Methods: Between 2000 and 2019, 187 Mutars prostheses were implanted in two dedicated centers: 72 upper limbs and 115 lower limbs reconstructions. Diagnoses included 107 primary malignant bone or soft tissue tumors, 65 metastases, 8 benign bone tumors and 7 non-oncologic cases. Silver-coated prostheses were used in 118/187 (63%) cases., Results: At last follow-up, 76.5% of patients had retained their implant. The overall failure rate was 23.5% at a mean of 1.7 years. There were 22 mechanical failures and 22 non-mechanical failures. The overall implant survival to all types of failure was 68% and 52% at 5 and 10 years, respectively. Infection was the most common mode of failure with an incidence of 6.9%. Implant survival to infection was better for silver-coated implants than for standard implants even if with no significant difference (p = 0.56). Functional results were satisfactory in 97% of patients., Conclusions: The overall implant survival at long term was satisfactory with Mutars prostheses. The incidence of complications with Mutars prosthesis is in line with the incidence reported in the literature with other types of tumor prosthesis. The most frequent cause of failure was infection with a lower incidence in silver-coated prostheses; silver coating seems to prevent infection in distal femur and proximal tibia. The silver coating seems to be particularly useful in two-stage revisions with a lower incidence of secondary amputation. In higher risk patients, silver-coated prostheses are the preferable choice for the reduction of the reinfection rate. The functional results of Mutars prostheses were excellent or good in most of cases. The current paper is design to enhance the literature on megaprosthesis in tumor surgery, proven that this system is one of the most used all over the word and one of the best performing., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)

Introduction: The role of chemotherapy for patients with dedifferentiated chondrosarcoma (DDCS) is still under discussion. Here, we present the outcome in patients with DDCS treated with intensive chemotherapy from the EUROpean Bone Over 40 Sarcoma Study., Materials and Methods: The chemotherapy regimen included doxorubicin, ifosfamide and cisplatin. Postoperative methotrexate was added in case of poor histological response. Toxicity was graded based on the National Cancer Institute expanded common toxicity criteria, version 2.0, and survival was analysed using Kaplan-Meier curves, log-rank tests and univariate Cox regression models., Results: Fifty-seven patients with DDCS (localised, 34 [60%]; metastatic, 23 [40%]) aged 42-65 years were included. Surgical complete remission (SCR) was achieved in 36 (63%) patients. The median overall survival (OS) was 24 months (95% confidence interval, 22-25), and the 5-year OS was 39%. Patients with extremity localisation had a 5-year OS of 49% compared with 29% in patients with a central tumour (P = 0.08). Patients with localised disease had a 5-year OS of 46%, whereas patients with metastatic disease had a 5-year OS of 29% (P = 0.12). Patients in SCR had a 5-year OS of 49%, whereas patients not in SCR had a 5-year OS of 23% (P = 0.004). Chemotherapy toxicity was considerable but manageable. There was no treatment-related death, and 39 (70%) patients received ≥6 cycles of the planned nine chemotherapy cycles., Conclusions: Adding intensive chemotherapy to surgery for treatment of DDCS is feasible and shows favourable survival data compared with previous reports. With the limitations of data from a non-controlled trial, we conclude that chemotherapy could be considered in the management of patients aged >40 years., Competing Interests: Conflict of interest statement The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: S.B. has reported grants from Deutsche Krebshilfe, Deutsche Forschungsgemeinschaft and the European Science Foundation and personal fees from Lilly, Bayer, Pfizer, Novartis, Isofol and Clinigen; E.P. has reported advisory roles for Amgen, Daiichi Sankyo, Eli Lilly, EUSA Pharma and Deciphera, received research support from Bristol Myers Squibb, Pfizer and PharmaMar and received travel support from Lilly, PharmaMar and Takeda; L.K. has reported honoraria from Bayer; P.R. has reported advisory roles for Exactech and Stryker; A.F. has reported honoraria from Ipsen Pharma, PharmaMar and Lilly; P.C. has reported honoraria for the speaker, consultancy or advisory role from Bayer, Deciphera, Eisai, Eli Lilly, Nektar Therapeutics and Pfizer and received research funds from Advenchen Laboratories, Amgen Dompé, AROG Pharmaceuticals, Bayer, Blueprint Medicines, Daiichi Sankyo, Deciphera, Eisai, Eli Lilly, Epizyme Inc., Glaxo, Karyopharm Pharmaceuticals, Novartis, Pfizer and PharmaMar; P.R. has reported honoraria from Bayer, Clinigen, BMS, Roche, MSD, Deciphera, Novartis, Pfizer, PharmaMar, Lilly and Amgen; I.H., S.F., K.S.H., P.P., S.H.-N., D.M.D., C.B., B.B., E.St., M.G., T.K., M.E., V.F., M.K., R.B., D.B., O.B., A.C., R.S., R.B., T.K., L.H., G.A., B.-N.H., E.Se., A.F., F.K., G.H.-W. and S.S. have declared no conflicts of interest., (Copyright © 2021 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

An intraosseous lipoma is considered one of the rarest primary bone tumors. The etiology of this lesion remains unclear; many lesions are asymptomatic and appear only as incidental findings during routine radiographic evaluations. Magnetic resonance imaging of intraosseous lipomas can help to establish a diagnosis and to stage the neoplasm. This is a case report of a 53-year old man with a rare intraosseous lipoma of the patella.

Background: Three-dimensional (3D) printing is an emerging technology used in numerous medical fields. Reconstruction of large bone defects after tumor resections or complex revision surgeries is challenging especially in specific sites where modular prostheses are not available. The possibility to realize custom-made 3D-printed prostheses improves their application in surgical field despite the complication rate, gaining a lot of attention for potential benefits., Objectives: We asked: (1) What are the emerging indications and designs of 3D-printed prostheses for complex bone reconstructions? (2) What complications occur with the use of custom implants considering site?, Study Design and Methods: We performed a retrospective analysis of every patient in whom a custom-made 3D-printed prosthesis was used to reconstruct a bone defect after resection for a bone tumour or challenging revision surgery from 2009 to 2018 in two referral centres. Forty-one patients (11 males [27%], 30 females [73%]) with a mean age of 41 years (range, 10-78 years) were included. Our general indications for using these implants were complex reconstructions of massive bone defects, in the absence of available modular prostheses. Seven were non-oncologic patients, whereas 24 patients were mainly treated for their malignant bone tumours. Custom-made 3D-printed prostheses were used in pelvis (29), forearm (6), scapula (2), distal tibia (2), calcaneus (1), and femoral diaphysis (1). The reconstruction included complete articular replacement in 24 cases (58%) whereas a combined spinopelvic implant has been used in two cases. Flaps were used in 25 cases (61%). Statistical analyses include Kaplan-Meier curves of survival., Results: The mean follow-up was 20 months. In the oncologic group, overall survival was 89% at five year follow-up and only three patients died of disease. Only one patient required implant removal due to deep infection. Overall major and minor complication rate was 22% (14 complications in 9/41 patients), mainly wound-related problems. One patient reported a periprosthetic fracture, one had hip dislocation, and four (12% [4/34 cases]) had local recurrence. Mean MSTS functional outcome score at follow-up was 73% (range, 23-100%), with a full weight bearing at an average time of 73 days from surgery of lower limbs., Conclusions: Custom-made 3D-printed prostheses represent at today a promising reconstructive technique, maintaining however the correct indications for their use in musculoskeletal oncology and challenging revision surgery. Complication rate is acceptable, with infection and wound healing problems relatively common after complex pelvic reconstructions. We will continue to follow our patients over the longer term to ascertain the role of these implants; however, larger studies will need to confirm indications and control for prognostic factors.

The spinal column represents the third most common site for metastases after the lungs and the liver, and the most common site for metastatic bone disease. With life-extending advances in the systemic treatment of cancer patients, the surgical procedures performed for spinal metastases will increase, and their related complications will increase unavoidably. Furthermore, considering the high complication rates reported in the spinal literature regarding spine surgery overall, it becomes clear that a better understanding of complications that the cancer patients with spinal metastases may experience is necessary. This article aims to summarize and critically examine the current evidence for complications after spine surgery for metastatic spinal disease, in both the perioperative and postoperative period. This paper would be useful for the treating physicians of these patients in their clinical practice.

Background: After proximal humerus resection for bone tumors, restoring anatomy and shoulder function remains demanding because muscles and bone are removed to obtain tumor-free surgical margins. Current modes of reconstruction such as anatomic modular prostheses, osteoarticular allografts, or allograft-prosthetic composites and arthrodeses are associated with relatively poor shoulder function related to loss of the deltoid and rotator cuff muscles. Newer prosthetic designs like the reverse total shoulder arthroplasty (RTSA) are felt to be useful in other reconstructions where rotator cuff function is compromised, so it seemed logical that it might help in tumor reconstructions as well in patients where the deltoid muscle and its innervation can be preserved., Questions/purposes: In patients with a tumor of the proximal humerus that can be resected with preservation of the deltoid muscle, (1) What complications are associated with tumor resection and reconstruction with a modular RTSA? (2) What are the functional results of modular RTSA in these patients?, Methods: From January 2011 to January 2018, we treated 52 patients for bone tumors of the proximal humerus. Of these, three patients were treated with forequarter amputation, 14 were treated with standard modular proximal humerus implants, seven were treated with allograft-prosthetic composites (RTSA-APC), and 28 were treated with a modular RTSA. Generally, we used anatomic modular prosthetic reconstruction if during the tumor resection none of the abductor mechanism could be spared. Conversely, we preferred reconstruction with RTSA if an innervated deltoid muscle could be spared, but the rotator cuff and capsule could not, using RTSA-APC or modular RTSA if humeral osteotomy was distal or proximal to deltoid insertion, respectively. In this study, we retrospectively analyzed only patients treated with modular RTSA after proximal humerus resection. We excluded three patients treated with modular RTSA as revision procedures after mechanical failure of previous biological reconstructions and three patients treated after December 2016 to obtain an expected minimum follow-up of 2 years. There were nine men and 13 women, with a mean (range) age of 55 years (18 to 71). Reconstruction was performed in all patients using silver-coated modular RTSA protheses. Patients were clinically checked according to oncologic protocol. Complications and function were evaluated at final follow-up by the treating surgeon (PR) and shoulder surgeon (AC). Complications were evaluated according to Henderson classification. Functional results were assessed with the Musculoskeletal Tumor Society score (range 0 points to 30 points), Constant-Murley score (range 0 to 100), and American Shoulder and Elbow Surgeons score (range 0 to 100). The statistical analysis was performed using Kaplan-Meier curves., Results: Complications occurred in five of 22 patients; there was a shoulder dislocation (Type I) in four patients and aseptic loosening (Type II) in one. Function in these patients on the outcomes scales we used was generally satisfactory; the mean Musculoskeletal Tumor Society score was 29, the mean Constant score was 61, and the mean American Shoulder and Elbow Surgeons score was 81., Conclusions: Although this was a small series of patients with heterogeneous diagnoses and resection types, and we were not able to directly compare the results of this procedure with those of other available reconstructions, we found patients treated with RTSA achieved reasonable shoulder function after resection and reconstruction of a proximal humerus tumor. It may not be valuable in all tumor resections, but in patients in whom the deltoid can be partly spared, this procedure appears to reasonably restore short-term shoulder function. However, future larger studies with longer follow-up are needed to confirm these findings., Level of Evidence: Level IV, therapeutic study.

Giant cell reparative granulomas (GCRGs) are non-neoplastic inflammatory lesions, usually observed in the maxilla, mandible or small bones of the hands and feet. These lesions present a wide range of morphology and the misinterpretation with other giant cell lesions can often occur. We report the case of a 47-year-old woman with GCRG in the left scapula, presenting some uncommon features: the location (scapula) and age at presentation, the lack of underlying bone disease such as Paget's disease or fibrous dysplasia, the large aggressive expansile aspect of the lesion. This was a therapeutic study, level IV (case series with no or a historical control group).

Objective: Infection after orthopaedic oncology surgery is a relatively frequent complication. Infection rate ranges in the literature between 3.7% and 19.9%, increasing up to 47% after pelvic resection and reconstruction. It represents a challenging topic when occurring in oncologic patients because of the delay of systemic and local treatments, influencing prognosis. Infection is a major concern in terms of both prevention and treatment. The aim of our review was to analyze data reported in the literature about strategies and new materials for infection prevention in musculoskeletal oncology surgery., Materials and Methods: We reviewed the literature focusing on the use of new materials that can reduce the risk of infection, avoiding biofilm formation on the implant surface., Results and Discussion: New materials are available to try to reduce the risk of infection. Iodine-coating, DAC-coating or silver-coating, are the more promising technologies available at today. Initial results with DAC-coating in non-oncological patients are interesting; however, studies about its efficacy in preventing infection in orthopaedic oncology are not present in literature. On the other side, iodine-coating implants or silver-coating prostheses demonstrated efficacy against early infections, associated with lower risk of implant removal and amputation as final surgery., Conclusions: Post-operative infections in orthopaedic oncology surgery are still frequent, and their diagnosis and treatment are demanding. According to the literature, silver-coated prostheses should be considered as the best option in case of revision surgery due to infection. However, there is no evidence that these new materials are effective to decrease the risk of infection drastically. Further studies with numerous series and long-term follow up are required.

A concept consolidated in recent years is that tumor growth depends to a great extent on the micro-environment surrounding the tumor, which has a fundamental role in tumor progression and in determining the effectiveness of therapies. Our analysis focuses on chondrosarcoma, the second primary malignant bone tumor, resistant to both chemotherapeutic and radiation therapy. We quantified hyaluronan, one of the main components of the extracellular matrix, with the aim of comparing its amount in the connective tissue surrounding the tumor with intra-tumoral tissue and healthy fascia of the same anatomic district, viewed as a health control. We demonstrate that hyaluronan increased significantly in the peritumoral stroma compared with the healthy fascia, which showed an average amount according to the physical characteristics of body districts by a mean value of 26.9 μg/g. In the peritumoral stroma, the mean hyaluronan content reached 132.6 μg/g (mean value of 63.2 μg/g). The p-value was less than 0.01, showing a highly significant statistical difference. Surprisingly, no significant differences were detected as a function of age, gender, or tumor grade. The levels of hyaluronan were comparable in peritumoral and tumor tissues, although there were differences depending on the state of necrosis. In addition, data on the expression of hyaluronic acid synthetase showed a decrease of about 50% in peritumoral and tumor tissues, indicating alterations in hyaluronan turnover and synthesis. This work demonstrates a variation in hyaluronan contents around the chondrosarcoma, likely correlated with the aggressiveness and resistance to chemotherapy of this tumors. Statement of Clinical Significance: Deeper knowledge about the composition of the peritumoral stroma, rich in extracellular matrix, will enhance better study and understanding of the metastatic potential of tumors and their prognostic indices. © 2018 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 37:503-509, 2019., (© 2018 Orthopaedic Research Society. Published by Wiley Periodicals, Inc.)

Background: The use of custom-made 3D-printed prostheses for reconstruction of severe bone defects in selected cases is increasing. The aims of this study were to evaluate (1) the feasibility of surgical reconstruction with these prostheses in oncologic and non-oncologic settings and (2) the functional results, complications, and outcomes at short-term follow-up., Methods: We analyzed 13 prospectively collected patients treated between June 2016 and January 2018. Diagnoses were primary bone tumour (7 patients), metastasis (3 patients), and revision of total hip arthroplasty (3 patients). Pelvis was the most frequent site of reconstruction (7 cases). Functional results were assessed with MSTS score and complications according to Henderson et al. Statistical analysis was performed using Kaplan-Meier and log-rank test curves., Results: At a mean follow-up of 13.7 months (range, 6-26 months), all patients except one were alive. Oncologic outcomes show seven patients NED (no evidence of disease), one NED after treatment of metastasis, one patient died of disease, and another one was alive with disease. Overall survival was 100% and 80% at one and two years, respectively. Seven complications occurred in five patients (38.5%). Survival to all complications was 62% at two years of follow-up. Functional outcome was good or excellent in all cases with a mean score of 80.3%., Conclusion: 3D-printed custom-made prostheses represent a promising reconstructive technique in musculoskeletal oncology and challenging revision surgery. Preliminary results were satisfactory. Further studies are needed to evaluate prosthetic design, fixation methods, and stability of the implants at long-term.

Background: Metastatic lesions to the proximal femur occur frequently and require special consideration due to the high risk of pathologic fractures. Type of surgery might influence patient survival considering the growing concept of oligometastases. In fact, the use of modular tumor megaprosthesis is increasing in the last decades compared to intramedullary nailing. Aim of this study was to evaluate oncological and functional results of treatment in patients with pathologic or impending fracture of the proximal femur, with patient survival being the primary, complications the secondary, and functional results the tertiary endpoint., Methods: Between 2016 and 2017, 40 patients with pathologic fracture (29 cases) or impending fracture according to the Mirels score (11 cases) of the proximal femur, were treated in our Institute and prospectively collected. There were 29 females (72.5%) and 11 males (27.5%), with a mean age at diagnosis of the metastasis of 63.6 years (range 35 to 92 years). Patients were treated due to bone metastases (commonly develop from breast cancer) or hematologic malignancies. Considering number of lesions, 17 patients had less than three bone metastases. Surgical procedures included intramedullary nailing (7 patients), conventional endoprosthesis (4 patients) and modular endoprosthetic replacement (29 patients). Adjuvant treatments included chemotherapy (13 cases), radiation therapy (8 cases) or both (15 cases), and selective arterial embolization (6 pre-op). Oncological results were evaluated considering the survival of patients. Functional results were assessed as pain intensity in VAS score and MSTS score., Results: The mean follow-up of patients was 10.2 months (range 6-26.3 years). At the latest evaluation, 23 patients were alive with disease, 3 patients were alive without evidence of disease and 14 patients were dead with disease. There was a significant better survival in patients treated with PFR compared to IMN and EPR groups (p = 0.0080). No differences in term of survival were found comparing impending vs actual pathological fracture and oligo vs multiple metastases. After surgery, all patients experienced improvement in quality of life resulting from reduction in pain. Mean MSTS score was 22.4. The overall complications rate was 22.5%. The most frequent complication was dislocation followed by wound dehiscence and deep infections., Conclusion: Modular tumour prosthesis for proximal femur replacement provides good functional outcome, relative low incidence of complications and higher life quality in the medium term. Oncologic results were influenced by type of surgery, biased by the correct indications for resection and nailing. Preoperative general health condition, life expectancy and ambulatory capacity may influence treatment strategy. With the numbers available, the patients with actual pathologic or impending fracture of the proximal femur treated with resection had a significantly higher survival, especially those with metastases from renal carcinoma or multiple myeloma., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Chondroblastoma (CB) of the foot is a rare lesion. The purpose of this report was to report a large series from a single institution to analyze clinical, radiographic, and histologic characteristics; treatment; and local recurrence. We present 40 patients (30 males, 10 females; mean age 25 years) diagnosed and treated for CB of the foot from 1975 to 2012. The mean follow-up visit was 55 months (range 7 months to 11 years). Clinical presentation, histology, imaging, surgical treatment, and local recurrence were evaluated. Males were more affected than females. The main symptom was pain (100%) accompanied by swelling (35%), with median duration of 12 months. The talus (50%) and calcaneus (37.5%) were the most affected bones. All patients underwent surgery: curettage (10 cases), curettage and bone graft (15 cases), curettage and cement (13 cases), wide resection (1 case), and Chopart amputation (1 case). Ten patients (25%) had secondary aneurysmal bone cyst. One patient had local recurrence after surgery. In conclusion, patients with CB of the foot are usually older than 20 years, and males are most affected. The hindfoot is the most affected area. Surgical treatment is required, and intralesional curettage and packing with cement or graft is curative in most cases. Local recurrence in foot is lower than in other locations., (Copyright © 2018. Published by Elsevier Inc.)

Background: The survival of patients with tumors around the shoulder treated with extra-articular resection, the rates of reconstructions-related complications, and the function of the shoulder cannot be estimated because of limited available data from mainly small published related series and case reports., Methods: We studied 54 patients with tumors around the shoulder treated with extra-articular shoulder resections and proximal humeral megaprosthetic reconstructions from 1985 to 2012. Mean tumor volume was 549 cm 3 , and the mean length of the proximal humeral resection was 110 mm. Mean follow-up was 7.8 years (range, 3-21 years). We evaluated the outcomes (survival, metastases, recurrences, and function) and the survival and complications of the reconstruction., Results: Survival of patients with malignant tumors was 47%, 38%, and 35%, at 5, 10, and 20 years, respectively. Rates for metastasis and local recurrence were 60% and 18.5%, respectively. Survival was significantly higher for patients without metastases at diagnosis, tumor volume <549 cm 3 , and type IV resections. Survival of reconstructions was 56% at 10 years and 48% 20 years. Overall, 19 patients (35.2%) experienced 30 complications (55.5%), the most common being soft tissue failures that required subsequent surgery without, however, implant removal. The mean Musculoskeletal Tumour Society score was 25 points, without any significant difference between the types of extra-articular resections., Conclusion: Tumor stage and volume as well as type of resection are important predictors of survival of patients with malignant tumors around the shoulder. Survival of the reconstructions is satisfactory; nevertheless, the complication rate is high. The Musculoskeletal Tumour Society score is similar with respect to the type of resection., (Copyright © 2017 Journal of Shoulder and Elbow Surgery Board of Trustees. Published by Elsevier Inc. All rights reserved.)

Purpose: To analyze the clinical and radiographic characteristics, treatment, and outcome of patients with elbow osteoblastoma., Patients and Methods: We present 13 patients (7 males and 6 females; mean age, 28 years) diagnosed and treated for an elbow osteoblastoma from 1975 to 2012. Mean follow-up was 29 months (range 12-60 months). Clinical presentation, imaging, surgical treatment, complications, range of elbow motion, and functional outcome were evaluated. The MSTS, DASH, and OXFORD scores were used., Results: Main symptom was pain (all patients) accompanied by stiffness (8 patients) and swelling or tumefaction (7 patients), with a median duration of symptoms of 32 months (range 6-96 months). Distal humerus was affected in 10 patients, proximal ulna in 2 patients, and proximal radius in one patient. All patients underwent surgical therapy that consisted of curettage of the lesion (7 patients), curettage and bone allografting (3 patients), wide resection (2 patients; total distal humerus and resection of the radial head), and radiofrequency thermal ablation (1 patient). One patient experienced a recurrence after surgical treatment. The mean MSTS score after treatment was 87% (range 50-100%), which corresponds to excellent results., Conclusions: Intralesional surgery is successful in tumor control in most patients with osteoblastoma of the elbow. Thermal ablation may be successful for smaller lesions. Most of the patients had a good-to-excellent functional outcome even if they had tumor-related elbow stiffness at diagnosis., Level of Evidence: Therapeutic study, Level IV-1.

Purpose: Chondroblastoma (CB) is a rare benign tumor that occurs most commonly in the second decade of life. No studies on CB in adulthood have been reported. Our purposes were to report a single-institution experience on CB in adults and to discuss the clinical and imaging findings, type of treatment, oncologic and functional outcomes., Patients and Methods: All patients diagnosed and treated for CB from 1981 to 2014 were reviewed. The main inclusion criterion was patients above their 30 years of age at diagnosis. Clinical presentation, imaging, surgical treatment, complications and functional outcome were evaluated. Local recurrences were recorded., Results: There were 15 males (65%) and 8 females (35%) with a mean age of 40 years (range 30-59 years). Tumor involved the small bones (talus, calcaneus, acromion, cuneiform, metatarsals) in 15 patients (65%) and the long bones in eight patients (35%). Treatment consisted of curettage only (6 cases), curettage plus phenol (13 cases), curettage plus radiotherapy (one patient with vertebral tumor), resection (two patients with CB of the acromion) and chopart amputation (one patient with a metatarsal tumor). At a mean follow-up of 8.4 years (range 3-30 years), twenty patients (87%) remained continuously disease-free, whereas three had local recurrences. None of the patients received chemotherapy., Conclusion: This study confirms that the occurrence and outcome of adult patients with CB are similar with those affecting younger patients. The main difference consists of the involved bones; flat bones and short tubular bones of the foot, especially the talus and calcaneus, are most commonly involved as opposed to long tubular bones., Level of Evidence: Therapeutic study, level IV-1 (case series).

An intercalary reconstruction is defined as replacement of the diaphyseal portion of a long bone after segmental skeletal resection (diaphysectomy). Intercalary reconstructions typically result in superior function compared to other limb-sparing procedures as the patient's native joints above and below the reconstruction are left undisturbed. The most popular reconstructive options after segmental resection of a bone sarcoma include allografts, vascularized fibula graft, combined allograft and vascularized fibula, segmental endoprostheses, extracorporeal devitalized autograft, and segmental transport using the principles of distraction osteogenesis. This article aims to review the indications, techniques, limitations, pros and cons, and complications of the aforementioned methods of intercalary bone tumor resections and reconstructions in the context of the ever-growing, brave new field of limb-salvage surgery.

Tumors of the hand comprise a vast array of lesions involving skin, soft tissue and bone. The majority of tumors in the hand are benign. Malignant tumors, although rare, do occur and frequently have unique characteristics in this specific anatomic location. Careful staging, histological diagnosis and treatment are essential to optimize clinical outcome. However, straightforward most of the time, hand tumor management does have pitfalls; caution is advised, as a missed or delayed diagnosis or an improperly executed biopsy may have devastating consequences. This article reviews the clinical spectrum of the most common benign and malignant bone and soft tissue tumors of the hand and discusses the clinicopathological findings, imaging features and current concepts in treatment for these tumors.

Aims: Fibrocartilaginous mesenchymoma is a rare intraosseous lesion, with a total of 26 cases described in the literature. This study describes the clinical, radiological and histological features of eight new cases of fibrocartilaginous mesenchymoma collected at a single institution between 1982 and 2016. The presence of GNAS and IDH1/2 mutations and MDM2 amplification was explored to evaluate possible links between fibrocartilaginous mesenchymoma, fibrous dysplasia, de-differentiated chondrosarcoma and low-grade osteosarcoma., Methods and Results: Eight new cases of fibrocartilaginous mesenchymoma of bone identified in our archives, dating from 1982 to 2016, were reviewed. The diagnosis was not performed on the initial biopsy in any of these cases, due mainly to the absence of obvious cartilaginous differentiation. On imaging, the tumour contained cartilaginous calcifications and showed a very strong uptake of contrast medium after injection. Histologically, the tumour was characterized by spindle cell proliferation mimicking a low-grade spindle cell sarcoma, associated with epiphyseal growth-plate-like nodules of cartilage and bone production. Molecularly, no GNAS and IDH1/2 mutations or MDM2 amplification were found in the cases analysed. Only one case recurred 1 year following intralesional resection. None died of disease., Conclusions: This very rare bone tumour has a typical radiological and histological pattern and a favourable survival outcome after treatment. Local recurrences can be prevented with complete surgery. Fibrocartilaginous mesenchymoma does not seem to be related genetically to fibrous dysplasia, low-grade osteosarcoma and de-differentiated chondrosarcoma., (© 2017 John Wiley & Sons Ltd.)

The introduction of multidisciplinary approach with chemo and radiotherapy, the advances in surgical and the improvements of diagnostic techniques allowed limb salvage surgery in most cases of bone sarcomas instead of amputation. Modular megaprostheses are the most common method of reconstruction after segmental resection of the long bones in the extremities for their availability, immediate fixation, early weight bearing, good function. Despite the advances in materials and implant designs, these systems have an high incidence of complications. Aim of this study was to report the experience on mega-prostheses implanted around the knee in tumor and revision surgery to analyze: the most frequent used current systems, the problems of stems fixation, extensor mechanism reconstructions in proximal tibia resections and the preservation of growth of the lower extremity in children.

Purposes: Telangiectatic osteosarcoma (TOS) is a rare subtype of osteosarcoma. We analyzed (1) oncologic outcome in a large homogeneous series and (2) the role of prognostic factors on prognosis, local recurrence and metastasis., Methods: Eighty-seven patients (47 males, 54 %) were retrospectively analyzed. All except 4 had extracompartmental disease, and ten patients had lung metastasis at diagnosis. Pathologic fracture was present in 27 cases (31 %). Seventy-eight patients were treated with neoadjuvant chemotherapy; nine had surgery as first treatment. Limb-salvage surgery was performed in 71 cases, amputation in 14, and rotationplasty in one. One patient died before surgery. Possible prognostic factors were statistically evaluated., Results: Overall survival was 60.7 % at 10 years of follow-up. Fifty-one patients were disease-free (58.6 %), 2 were alive with disease (2.3 %), 31 died with disease (35.6 %), and 3 died of other causes (3.4 %). Ten local recurrences were observed (11 %). Twenty-five patients (29 %) developed lung (22) or bone (3) metastases. No statistical difference was found considering age, metastases at diagnosis, gender, pathologic fracture, tumor volume, compartmental status, number of neoadjuvant chemotherapy agents and treatment. Induced necrosis was significant at both univariate and multivariate analysis (p < 0.0001)., Conclusions: TOS does not have a poor prognosis as previously reported in literature, with a survival of about 60 % at 10 years. Most of patients can be cured with neoadjuvant chemotherapy plus surgery (limb sparing surgery is possible and safe). Tumor response to chemotherapy as induced necrosis was the only significant prognostic factors on survival, even if small tumor volume at diagnosis correlates with better prognosis at univariate analysis., Level of Evidence: IV.

Background: Metastatic disease is the most common malignancy of the bone. Prostate, breast, lung, kidney, and thyroid cancer account for 80% of skeletal metastases. Bone metastases are associated with significant skeletal morbidity including severe bone pain, pathologic fractures, spinal cord or nerve roots compression, and malignant hypercalcemia. These events compromise greatly the quality of life of the patients. The treatment of cancer patients with bone metastases is mostly aimed at palliation., Objective: This article aims to present these palliative treatments for the patients with bone metastases, summarize the clinical applications, and review the techniques and results., Methods: It gives an extensive overview of the possibilities of palliation in patients with metastatic cancer to the bone., Results and Discussion: Currently, modern treatments are available for the palliative management of patients with metastatic bone disease. These include modern radiation therapy, chemotherapy, embolization, electrochemotherapy, radiofrequency ablation, and high-intensity focused ultrasound. As such it is of interest for all physicians with no experience with these developments to make palliative procedures safer and more reliable.

Background: Infection of megaprostheses after bone tumor resection is a major concern; management is challenging. This study evaluated the survivability from such infections, the microbial isolates, treatment tactics, and outcome of megaprosthesis reconstructions., Materials and Methods: We studied 1,161 patients retrospectively who underwent megaprosthesis reconstruction for limb salvage after a sarcoma from 1983 to 2010. The mean followup was 9 y (range 3-20 y). We evaluated the overall survival of the megaprosthesis reconstructions in patients with infection and the survival with respect to the type of megaprosthesis, site of reconstruction, cemented or cementless fixation, type of tumor, adjuvant treatments, microbial isolate(s), treatment tactics, and outcome., Results: The incidence of infection was 8.6%. The most common microbial isolate was Staphylococcus epidermidis (47%). Overall survival with definitive management of infection was 88% at 10 y and 84% at 20 y. Survival was higher for cementless reconstructions and not different with respect to the type of megaprosthesis, site of reconstruction, or adjuvant therapy. Infections resolved completely with one- or two-stage surgery in 75% of patients. The rate of amputation because of infection was 21%., Conclusions: Megaprosthesis reconstructions may be infected in 8.6% of cases. Infections more commonly occur late, caused usually by S. epidermidis. The survival rate is higher with cementless megaprosthesis reconstructions and no different with respect to the type of tumor or megaprosthesis or the adjuvant treatments. One-stage revision is effective for acute post-operative infections; however, two-stage revision surgery is necessary for early and late infections. The rate of amputation because of occurrence or persistence of megaprosthesis infection is 21%.

Background: We recently developed a clinical decision support tool, capable of estimating the likelihood of survival at 3 and 12 months following surgery for patients with operable skeletal metastases. After making it publicly available on www.PATHFx.org , we attempted to externally validate it using independent, international data., Methods: We collected data from patients treated at 13 Italian orthopaedic oncology referral centers between 2010 and 2013, then applied to PATHFx, which generated a probability of survival at three and 12-months for each patient. We assessed accuracy using the area under the receiver-operating characteristic curve (AUC), clinical utility using Decision Curve Analysis (DCA), and compared the Italian patient data to the training set (United States) and first external validation set (Scandinavia)., Results: The Italian dataset contained 287 records with at least 12 months follow-up information. The AUCs for the three-month and 12-month estimates was 0.80 and 0.77, respectively. There were missing data, including the surgeon's estimate of survival that was missing in the majority of records. Physiologically, Italian patients were similar to patients in the training and first validation sets. However notable differences were observed in the proportion of those surviving three and 12-months, suggesting differences in referral patterns and perhaps indications for surgery., Conclusions: PATHFx was successfully validated in an Italian dataset containing missing data. This study demonstrates its broad applicability to European patients, even in centers with differing treatment philosophies from those previously studied.

Background: Modular megaprostheses are now the most common method of reconstruction after segmental resection of the long bones in the lower extremities. Previous studies reported variable outcome and failure rates after knee megaprosthetic reconstructions., Questions/purposes: The objectives of this study were to analyze the results of a modular tumor prosthesis after resection of bone tumor around the knee with respect to (1) survivorship; (2) failure rate; (3) comparative survivorship against different sites of reconstructions and of primary and revision implants; and (4) functional results on the Musculoskeletal Tumor Society (MSTS) scoring system., Methods: Between 2003 and 2010, 247 rotating-hinge Global Modular Reconstruction System (GMRS) knee prostheses were implanted in our institute for malignant and aggressive benign tumors. During this time, that group represented 23% of the patients who had oncologic megaprosthesis reconstruction about the knee after resection of primary or metastatic bone tumors (247 of 1086 patients). In the other 77% of cases we used other types of oncologic prostheses. Before 2003 we used the older Howmedica Modular Resection System and Kotz Modular Femur/Tibia Replacement from 2003 we used mostly the GMRS but we continued to use the HMRS in some cases such as patients with poor prognoses, elderly patients, or metastatic patients. Sites included 187 distal femurs and 60 proximal tibias. Causes of megaprosthesis failure were classified according to Henderson et al. in five types: Type 1 (soft tissue failure), Type 2 (aseptic loosening), Type 3 (structural failure), Type 4 (infection), and Type 5 (tumor progression). Followup was at a minimum oncologic followup of 2 years (mean, 4 years; range, 2-8 years). Kaplan-Meier actuarial curves of implant survival to major failures were done. Functional results were analyzed according to the MSTS II system; 223 of the 247 were available for functional scoring (81%)., Results: At latest followup, among 175 treated patients for primary reconstruction, 117 are continuously disease-free, 26 have no evidence of disease after treatment of relapse, eight are alive with disease, and 24 died from disease. The overall failure rate of the megaprostheses in our series was 29.1% (72 of 247). Type 1 failure occurred in 8.5% (21 of 247) cases, Type 2 in 5.6% (14 of 247), Type 3 in 0%, Type 4 in 9.3% (23 of 247), and Type 5 in 5.6% (14 of 247). Kaplan-Meier curve showed an overall implant survival rate for all types of failures of 70% at 4 years and 58% at 8 years. Prosthetic survivorship for revisions was 80% at 5 years and for primary reconstructions was 60% at 5 years (p = 0.013). Survivorship to infection was 95% at 5 years for revision patients and 84% at 5 years for primary patients (p = 0.475). The mean MSTS score was 84 (25.2; range, 8-30) with no difference between sites of localization (24.7 in proximal tibia versus 25.4 in distal femur reconstruction; p = 0.306)., Conclusions: Results at a minimum of 2 years with this modular prosthesis are satisfactory in terms of survivorship (both oncologic and reconstructive) and causes and rates of failure. Although these results seem comparable with other like implants, we will continue to follow this cohort, and we believe that comparative trials among the available megaprosthesis designs are called for., Level of Evidence: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

The objective of this study was to assess outcome and recurrence rate after limb-salvage surgery with reconstruction for pelvic bone tumors and analyze complications and their relationship with surgery. The authors analyzed 129 patients followed for a mean of 6 years (range, 2-19 years). Chondrosarcoma was the most frequent histotype. Thirty-one cases with no acetabular involvement were reconstructed with allograft only. Acetabular resections were reconstructed with allograft prosthetic composite (n=60), allograft only (n=11), trabecular metal components (n=2), prosthesis only (n=10), saddle prosthesis (n=11), and iliofemoral arthrodesis (n=1). Margins were wide (n=94), wide contaminated (n=22), marginal (n=7), and intralesional (n=6). Oncologic outcomes were as follows: 75 patients were continuously disease free, 6 were disease free after treatment of relapse, 13 were alive with disease, 28 were dead of disease, and 5 were dead of other causes. Survival was 66% at 10 years. Local recurrence rate of malignant tumors was 22.1% and was not statistically influenced by margins (P=.140) or site (P=.933). Metastasis rate was 32.8%. Deep infection was observed in 30 (23.6%) cases, with no statistical difference between reconstructions with and without allograft (P=.09). Final external hemipelvectomy was performed in 16 cases. Newer techniques of reconstruction using stemmed acetabular cups or porous metal components combined with allograft are now available. Local control and satisfactory survival is achievable long term in patients with pelvic tumors, but this surgery implies a high rate of complications. Infection is a major complication, not influenced by the use of allografts. Amputation is rarely needed., (Copyright 2015, SLACK Incorporated.)

Bone sarcomas are a variety of non-epithelial, malignant neoplasms of bone. The most common bone sarcomas are osteosarcoma, Ewing's sarcoma, and chondrosarcoma. The approach to a patient with a suspected bone sarcoma from initial examination to the histological diagnosis and classification is staging. Staging is of critical importance, in order to classify different treatment options and point out which combination of them is more suitable depending on the severity of the tumor in every individual patient. Staging should include medical history, physical and imaging examination, and biopsy. This article presents the current approach for staging, principles of biopsy, tumor classification, treatment, and follow-up of patients with bone sarcomas.

Background: The prognosis of patients with metastatic, recurrent, and/or unresectable osteosarcoma is poor. Aggressive local and medical treatments are available for palliation. Palliative treatments include isolated limb perfusion, radiation therapy, embolization, chemoembolization, thermal ablation, and cryoablation. Their aim is pain relief and tumor size reduction with minimum complications., Materials and Methods: We present 19 patients with metastatic, recurrent, and/or unresectable osteosarcoma of the pelvis and lower lumbar spine treated with palliative selective embolization using N-2-butyl cyanoacrylate. All patients had chemotherapy. At the time of embolization, they experienced severe pain refractory to analgesics. Diagnostic angiography was performed pre-embolization to determine the vascular mapping and hemodynamic status of the tumor. Post-embolization angiography was done to evaluate for complete occlusion of the pathological vessels. Mean follow-up was 18 months. Local pain, tumor necrosis and size, and complications were recorded., Results: In all patients, pre-embolization angiography showed hypervascularity of the tumor from extensive neovascularization. Five patients had repeat embolization. All patients experienced pain relief at a mean of 3 days post-embolization. No patient had recurrent pain with the intensity of that before embolization. Variable tumor necrosis was observed in follow-up imaging, and reduction in tumor size was minimum. All patients experienced pain at the site of embolization, which resolved completely 1-5 days after embolization. Four patients with pelvic osteosarcomas experienced paraesthesias at the distribution of the sciatic nerve., Conclusion: Selective arterial embolization is a useful local palliative treatment for patients with advanced osteosarcoma for pain relief.

Background: Angiosarcoma of bone is a rare high-grade malignant vascular tumor. The literature regarding treatment and outcome of patients with this tumor is limited.We performed a 2 institutional retrospective study to analyze treatment and survival of patients with angiosarcoma of bone., Patients and Methods: We reviewed patients with the histologic diagnosis of primary angiosarcoma of bone treated from 1980 to 2009. Demographic details, histology, treatment, and survival were reviewed., Results: A total of 38 men and 22 women (median age, 54 y) were recruited. Most lesions occurred in the femur and the pelvis. Metastatic disease at presentation was diagnosed in 24 patients (40%). Forty-three patients underwent surgery, with 30 of them achieving surgical complete remission (SCR). Radiotherapy was applied to 17 patients, and chemotherapy to 13/35 and 15/22 patients with localized and metastatic disease, respectively.The 5-year overall survival (OS) was 20%: 33% for patients with localized disease and 0% for metastatic patients. Higher 5-year OS was reported for patients who achieved SCR (46%) than for those who did not (0%). In nonmetastatic patients, a trend toward improved survival was observed after SCR and adjuvant chemotherapy based on cisplatin, doxorubicin, and ifosfamide.Fifteen patients received chemotherapy for metastases. Two RECIST partial responses of 13 evaluable patients were documented (paclitaxel [n=1] and doxorubicin [n=1]). Stable disease was observed in 2 patients., Conclusions: Complete surgical resection is essential for outcome. Survival of patients with metastatic or unresectable disease is very poor. Activity of taxanes and anthracycline was observed in the metastatic setting and merits further evaluation.

Previous classification systems of failure of limb salvage focused primarily on endoprosthetic failures and lacked sufficient depth for the effective study of the causes of failure. In order to address these inadequacies, the International Society of Limb Salvage (ISOLS) formed a committee to recommend revisions of the previous systems. The purpose of this study was to report on their recommendations. The modifications were prepared using an earlier, evidence-based model with subclassification based on the existing medical literature. Subclassification for all five primary types of failure of limb salvage following endoprosthetic reconstruction were formulated and a complementary system was derived for the failure of biological reconstruction. An additional classification of failure in paediatric patients was also described. Limb salvage surgery presents a complex array of potential mechanisms of failure, and a complete and precise classification of types of failure is required. Earlier classification systems lacked specificity, and the evidence-based system outlined here is designed to correct these weaknesses and to provide a means of reporting failures of limb salvage in order to allow the interpretation of outcome following reconstructive surgery., (©2014 The British Editorial Society of Bone & Joint Surgery.)

Background: For patients who have chondrosarcoma with unresectable disease, because of tumor location, tumor size, or extensive metastatic disease, treatment options are very limited because of their relative resistance to radiotherapy and chemotherapy. The overall survival of this patient population is poor; however, specific studies are lacking, and large series have not been published. Therefore, the authors conducted this retrospective, 2-center study to gain insight into the outcome of patients with advanced, unresectable, conventional central chondrosarcoma., Methods: All patients with unresectable conventional central chondrosarcoma who were diagnosed between January 1, 1980 and December 31, 2011 in 2 major European bone sarcoma centers (Rizzoli Institute, Bologna, Italy and Leiden University Medical Center, Leiden, the Netherlands) were selected. Relevant information was collected from the medical records at both centers., Results: In total, 171 patients met the selection criteria. The overall survival rate for all patients was 48% at 1 year, 24% at 2 years, 12% at 3 years, 6% at 4 years, and 2% at 5 years. Patients with unresectable, locally advanced disease without distant metastases had a significantly better survival than patients with metastatic disease (P = .0014). Systemic treatment, consisting of either doxorubicin-based chemotherapy or the noncytotoxic drugs imatinib and sirolimus, improved survival significantly compared with no treatment (P = .0487). For patients who had locally advanced disease without metastases, radiotherapy was associated with a survival benefit (P = .0032)., Conclusions: This study provides a standard for overall survival rates after a diagnosis of unresectable conventional central chondrosarcoma. Systemic treatment and radiotherapy may improve survival, although selection bias because of the retrospective nature of this study may have influenced the outcome. The poor survival underlines the need for new therapeutic options for this patient population. Cancer 2014;120:3159-3164. © 2014 American Cancer Society., (© 2014 American Cancer Society.)

The purpose of this article is to outline the current approach to patients affected by metastasis to the long bones and to present a clinical and surgical algorithm available for clinicians and for future research. A modern approach to patients affected by long bone metastasis in fact requires a multidisciplinary contest where oncologists, radiotherapists, surgeons and physical therapists cooperate with a shared vision, in order to provide the best possible integrated treatments available. The authors of this article constitute the Bone Metastasis Study Group of the Italian Orthopaedic Society (SIOT): a national group of orthopedic tumor surgeons who are dedicated to studying the approach, techniques and outcomes of surgery for metastatic tumours of the musculoskeletal system.

Objective: Purpose of this retrospective study was (1) to evaluate overall survival and survival to local recurrence of patients with unifocal and multifocal tumor and (2) to evaluate survival to local recurrence after intralesional or wide surgery., Material and Methods: We reviewed 62 patients with epithelioid hemangioendothelioma of bone, treated from 1985 to 2010. Histological sections and immunohistochemistry were evaluated. Tumor presented as unifocal in 49 patients and as multifocal in 13., Results: Mean follow-up was 9 years. Five patients (10%) with unifocal tumor progressed to multifocal. Overall survival was 92% at 10 years. Survival with unifocal tumor was significantly higher than survival with multifocal tumor. Survival to local recurrence was 76% at 10 years, significantly higher after wide resection than after intralesional surgery, while there was no statistical difference comparing unifocal and multifocal tumor. At multivariate analysis both variables showed no statistically significance., Conclusion: Wide surgical excision reduces the risk of local recurrence, but functional results and morbidity need to be considered individually when defining surgical indications. Due to the risk of radiation-induced sarcomas, radiation therapy should be reserved to those cases not amenable to wide surgery or when lesions are seated locations difficult to treat., (© 2014 Wiley Periodicals, Inc.)

Most tumors of the foot and ankle will be benign, and it has been easy for clinicians to underestimate the malignant potential of a given tumor. The purpose of our study was to gain insight into the incidence of different tumor types diagnosed in patients with tumors of the foot and/or ankle treated at the Rizzoli Institute from September 1990 to December 2007. Our series included 1170 patients and 1170 lesions, with an overall mean age at diagnosis of 43 (range 5 to 81) years. Overall, 870 lesions (74.36%) were nonmalignant and 300 (25.65%) were malignant. Soft tissue lesions were diagnosed in 189 patients (16.15%), of which 91 (48.15%) were nonmalignant (pseudotumors or benign). Bone lesions were diagnosed in 981 patients (83.85%), of which 779 (79.41%) were nonmalignant. Epidemiologic knowledge of foot abnormalities and clinical suspicion, an organized diagnostic method, and evidence-based treatment of musculoskeletal tumors are essential elements to obtaining optimal results in the treatment of malignant foot tumors., (Copyright © 2014 American College of Foot and Ankle Surgeons. Published by Elsevier Inc. All rights reserved.)

Study Design: Retrospective case series., Objective: (1) To analyze clinical and radiographical characteristics, treatment, and outcome in patients with sacral osteoblastoma, (2) to evaluate progression-free survival and local recurrence rate, and (3) to identify prognostic factors., Summary of Background Data: Osteoblastoma is a rare tumor that has been reported to affect the sacrum from 7% to 17%. Symptoms are various and the diagnosis is often delayed., Methods: From 1980 to 2010, 18 patients with sacral osteoblastoma (16 males and 2 females) were treated at Rizzoli Institute. Lesion involved S1 (2 cases), S1-S2 (3 cases), S2 (1 case), S2-S3 (1 case), S2-S4 (1 case), S3 (2 cases), S3-S4 (5 cases), S4 (1 case), and almost the entire sacrum in 2 cases. According to Enneking classification for benign bone tumors, 13 (72%) were diagnosed at stage 2 and 5 (28%) at stage 3. Mean tumor volume was 64 cm (range, 2-441 cm). Nine patients had preoperative magnetic resonance imaging. Five patients had a previous inadequate intralesional surgery elsewhere. Treatment consisted in intralesional surgery (16 cases), intralesional surgery and radiotherapy (1 case), and wide resection (1 case). Local adjuvants used were phenol (7 patients), cryocoagulation with "iceball" technique (1 case). Embolizations were performed in 7 patients., Results: At a mean of 8.4 years (range, 1-28 yr), 15 patients (83%) remained continuously disease free, whereas 3 patients had local recurrence (17%). Progression-free survival was 87% at 5 years and 74% at 10 years. No statistical difference was found between patients who received or not local adjuvants (P = 1.254), older or younger than 20 years (P = 0.970), at stage 2 or 3 (P = 0.826), evaluated preoperatively with or without magnetic resonance imaging (P = 0.160), primarily treated versus patients with previous intralesional surgery elsewhere (P = 0.131)., Conclusion: In our series, curettage was successful in most of the patients. Local adjuvants did not seem to reduce the risk of local recurrence when combined with intralesional surgery., Level of Evidence: 4.

Background: Surgical treatment of pelvic tumors with or without acetabular involvement is challenging. Primary goals of surgery include local control and maintenance of good quality of life, but the procedures are marked by significant perioperative morbidity and complications., Questions/purposes: We wished to (1) evaluate the frequency of infection after limb salvage surgical resection for bone tumors in the pelvis; (2) determine whether infection after these resections is associated with particular risk factors, including pelvic reconstruction, radiotherapy or chemotherapy, type of resection, and age; and (3) analyze treatment of these infections, particularly with respect to the need of additional surgery or hemipelvectomy., Methods: From 1975 to 2010, 270 patients with pelvic bone tumors (149 with chondrosarcoma, 40 with Ewing's sarcoma, 27 with osteosarcoma, 18 with other primary malignant tumors, 11 with metastatic tumors, and 25 with primary benign tumors) were treated by surgical resection. Minimum followup was 1.1 years (mean, 8 years; range, 1-33 years). The resection involved the periacetabular area in 166 patients. In 137 patients reconstruction was performed; in 133 there was no reconstruction. Chart review ascertained the frequency of deep infections, how they were treated, and the frequency of resection arthroplasty or hemipelvectomies that occurred thereafter., Results: A total of 55 patients (20%) had a deep infection develop at a mean followup of 8 months. There were 20 infections in 133 patients without reconstruction (15%) and 35 infections in 137 patients with reconstruction (26 %). Survivorship rates of the index procedures using infection as the end point were 87%, 83%, and 80% at 1 month, 1 year, and 5 years, respectively. Infection was more common in patients who underwent pelvic reconstruction after resection (univariate analysis, p = 0.0326; multivariate analysis, p = 0.0418; odds ratio, 1.7718; 95% CI, 1.0243-3.0650); no other risk factors we evaluated were associated with an increased likelihood of infection. Despite surgical débridements and antibiotics, 16 patients (46%) had the implant removed and five (9%) underwent external hemipelvectomy (four owing to infection and one as a result of persistent infection and local recurrence)., Conclusions: Infection is a common complication of pelvic resection for bone tumors. Reconstruction after resection is associated with an increased risk of infection compared with resection alone, without significant difference in percentage between allograft and metallic prosthesis. When infection occurs, it requires removal of the implant in nearly half of the patients who have this complication develop, and external hemipelvectomy sometimes is needed to eradicate the infection.

The appropriate diagnosis and treatment of bone tumors requires close collaboration between different medical specialists. Imaging plays a key role throughout the process. Radiographic detection of a bone tumor is usually not challenging. Accurate diagnosis is often possible from physical examination, history, and standard radiographs. The location of the lesion in the bone and the skeleton, its size and margins, the presence and type of periosteal reaction, and any mineralization all help determine diagnosis. Other imaging modalities contribute to the formation of a diagnosis but are more critical for staging, evaluation of response to treatment, surgical planning, and follow-up.When necessary, biopsy is often radioguided, and should be performed in consultation with the surgeon performing the definitive operative procedure. CT is optimal for characterization of the bone involvement and for evaluation of pulmonary metastases. MRI is highly accurate in determining the intraosseous extent of tumor and for assessing soft tissue, joint, and vascular involvement. FDG-PET imaging is becoming increasingly useful for the staging of tumors, assessing response to neoadjuvant treatment, and detecting relapses.Refinement of these and other imaging modalities and the development of new technologies such as image fusion for computer-navigated bone tumor surgery will help surgeons produce a detailed and reliable preoperative plan, especially in challenging sites such as the pelvis and spine., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Purpose: Managing patients with advanced bone sarcomas - namely, recurrent, unresectable and metastatic - is mostly aimed at palliation. The role of embolisation for pain relief for these patients has not been previously reported. We therefore performed this study to emphasise the palliative role of embolisation for pain relief of advanced bone sarcoma patients., Materials and Methods: We retrospectively studied 43 patients with advanced bone sarcomas treated with palliative embolisation with N-2-butyl-cyanoacrylate from 2004 to 2011. All patients had primary treatments including chemotherapy, radiation therapy, radiofrequency thermal ablation, and/or surgery for their advanced sarcomas and were referred for embolisation as end-stage treatment for continuous severe local pain. The effect of embolisation was evaluated with a pain score scale and analgesic use. Mean follow-up was 7 (range, 1-19) months); all patients were dead at the last follow-up., Results: In all patients, angiography showed increased pathological vascularisation of the sarcomas; three to six feeding vessels were embolised in each procedure. Almost complete pain relief and >50% reduction in analgesic use was experienced by 36 patients with highly hypervascular sarcomas and sarcomas in the pelvis and shoulder girdle. Moderate pain relief and 50% reduction in analgesic use was experienced by seven patients with spinal and sacral lesions. Within the available follow-up, no patient had recurrent pain with the same intensity as before embolisation. All patients experienced ischaemic pain at the site of embolisation that resolved completely with analgesics. Six patients with advanced pelvic bone sarcomas experienced paraesthesias at the distribution of the sciatic nerve that resolved completely with methylprednisolone., Conclusions: Embolisation is a safe and effective local palliative treatment for patients with advanced sarcomas, providing optimum pain relief with the least discomfort and the possibility of minor complications only.

Background: Patellar tumors are rare; only a few series have been described in the literature and radiographic diagnosis can be challenging. We reviewed all patellar tumors at one institution and reviewed the literature., Materials and Methods: In an evaluation of the database at one institution from 1916 to 2009, 23,000 bone tumors were found. Of these, 41 involved the patella. All had imaging studies and microscopic diagnostic confirmation. All medical records, imaging studies, and pathology were reviewed., Results: There were 15 females and 26 males, ranging from 8 to 68 years old (average 30). There were 30 benign tumors; eight giant cell tumors, eight chondroblastomas, seven osteoid osteomas, two aneurysmal bone cysts, two ganglions, one each of chondroma, exostosis, and hemangioma. There were 11 malignant tumors: five hemangioendotheliomas, three metastases, one lymphoma, one plasmacytoma, and one angiosarcoma., Conclusion: Patellar tumors are rare and usually benign. As the patella is an apophysis, the most frequent lesions are giant cell tumor in the adult and chondroblastoma in children. Osteoid osteomas were frequent in our series and easily diagnosed. Metastases are the most frequent malignant diagnoses in the literature; in our series malignant vascular tumors were more common. These lesions are often easily analyzed on radiographs. CT and MR define better the cortex, soft tissue extension, and fluid levels. This study presents the imaging patterns of the more common patellar tumors in order to help the radiologist when confronted with a lesion in this location., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Background and Objectives: Retrospectively analyze outcomes of current-generation Global Modular Replacement System (GMRS) modular tumor endoprosthesis for the lower limb in primary and secondary implantation procedures., Methods: Two hundred ninety five prostheses were implanted, 197 were primary implants, 98 were for revision surgery; revision procedures included 84 failed tumor reconstructions and 14 failed non-tumor reconstructions. Anatomic sites included: distal femur 199; proximal tibia 60; proximal femur 32;total femur 4. Endoprosthesis failures were classified as soft-tissue failures (Type 1), aseptic loosening (Type 2), structural fracture (Type 3), infection (Type 4), and tumor recurrence (Type 5). MSTS functional scores were measured., Results: The overall failure rate was 28.8% and failure occurred at a median of 1.7 years (range, 1 month to 7 years). At a mean oncologic follow up of 4.2 years (range, 2-8 years), 195 patients are continuously NED, 43 NED after treatment of relapse, 10 AWD, 33 DWD. There was a significant difference in implant survival of all modes of failure between primary and revision implants (P = 0.03). No prosthetic fracture occurred. The average functional score was 81.6% (24.5)., Conclusions: Mid-term results with GMRS are promising, with good functional results and low incidence of complications for primary implants., Level of Evidence: Therapeutic study, level IV-1 (case series)., (© 2013 Wiley Periodicals, Inc.)

Background: Knee arthrodeses are performed to treat infection after arthroplasty and tumors requiring extensive soft tissue resection. Many techniques have been described, but most have important disadvantages. Currently, endoprosthetic arthrodesis implants are available, but little is known about them., Questions/purposes: Our objective was to analyze a series of knee arthrodeses with a modular prosthetic system to evaluate (1) survivorship of the implant, (2) complications, (3) whether survivorship differed between arthrodeses done for primary tumor resection and revision reconstructions, and (4) whether survivorship differed based on the presence of previous infection or the use of a gastrocnemius flap., Methods: We present 32 patients with modular knee arthrodeses; arthrodeses were performed in 25 patients with tumors and in seven patients without tumors. There were 14 implants done at the time of tumor resection and 18 for revision of failed implants. Survivorship and complications were analyzed with Kaplan-Meier curves. Log-rank test was used for comparison between primary and revision implants, not infected and previously infected implants, and use or nonuse of a gastrocnemius flap., Results: Survivorship of arthrodeses with modular endoprostheses was 50% and 25% at 5 and 10 years, respectively. There were nine infections (29%) and one implant fracture (3%). Amputation as final surgery was required in 8 patients (six owing to infection and two to oncologic failures). There was no significant difference in survivorship between arthrodeses done for primary tumor resection and as a salvage procedure for failed implants. No differences were found between patients with and without prior infection or with and without a gastrocnemius flap., Conclusions: Survivorship of a modular arthrodesis implant was 50% at 5 years owing to a high complication rate. Infection is the most common cause of failure of oncologic and revision implants. Implant fracture is a rare occurrence. Modular segmental arthrodesis provides a stable construct for patients in whom limb preservation is possible but a hinged device is contraindicated as a result of major muscle resection., Level of Evidence: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Background: Studies for patients with pelvic chondrosarcomas are limited. This study determines the outcome of patients with pelvic chondrosarcomas, and whether there is any association with tumors' grade, type, stage, margins and pelvic location., Materials and Methods: We retrospectively studied 215 patients with pelvic chondrosarcomas. All patients had biopsy and histological diagnosis of their tumors followed by limb salvage or amputation. We staged patients using the Musculoskeletal Tumor Society system. We performed a univariate and multivariate analysis of the survival to death, local recurrence and metastasis with respect to grade, type (central vs. peripheral), stage, margins, and pelvic location, and the survival to death of patients with and without local recurrence., Results: Grade was the most important univariate and multivariate predictor of the survival of the patients. Dedifferentiation was associated with significantly lower overall survival. Peripheral chondrosarcomas predicted survival only in the univariate analysis. Surgical margins predicted local recurrence only in the multivariate analysis. Periacetabular location was associated with lower survival to death and local recurrence. The occurrence of local recurrence compromises the overall survival., Conclusion: Grade was the most important predictor of the overall survival of patients with chondrosarcomas of the pelvis., (Copyright © 2013 Wiley Periodicals, Inc.)

Purpose: This study was done to evaluate embolisation for palliative and/or adjuvant treatment of bone metastases from renal cell carcinoma and discuss the clinical and imaging results., Materials and Methods: We retrospectively studied 107 patients with bone metastases from renal cell carcinoma treated from December 2002 to January 2011 with 163 embolisations using N-2-butyl cyanoacrylate (NBCA). Mean tumour diameter before embolisation was 8.8 cm and mean follow-up 4 years. Clinical and imaging effects of treatment were evaluated at follow-up examinations with a pain score scale, analgesic use, hypoattenuating areas, tumour size and ossification., Results: A clinical response was achieved in 157 (96%) and no response in six embolisations of sacroiliac metastases. Mean duration of clinical response was 10 (range 1-12) months. Hypoattenuating areas resembling tumour necrosis were observed in all patients. Variable ossification appeared in 41 patients. Mean maximal tumour diameter after embolisation was 4.0 cm. One patient had intraprocedural tear of the left L3 artery and iliopsoas haemorrhage and was treated with occlusion of the bleeding vessel with NBCA. All patients had variable ischaemic pain that recovered completely within 2-4 days. Postembolisation syndrome was diagnosed after 15 embolisations (9.2%). Transient paraesthesias in the lower extremities were observed after 25 embolisations (25%) of pelvis and sacrum metastatic lesions., Conclusions: Embolisation with NBCA is recommended as primary or palliative treatment of bone metastases from renal cell carcinoma. Strict adherence to the principles of transcatheter embolisation is important to avoid complications.

Background: Previous studies reported variable outcome of proximal tibial resections and reconstructions. Therefore, we evaluated the survival, Musculoskeletal Tumor Society (MSTS) function, and complications of patients and reconstructions in this location., Materials and Methods: We reviewed the files of 225 patients with proximal tibial tumors treated with proximal tibial resection, fixed or rotating hinge megaprosthetic reconstruction and extensor mechanism reattachment using sutures, mechanical clamping, artificial ligaments, and/or gastrocnemius flap from 1985 to 2010 (mean follow-up, 90 months; median, 56; range, 2-294). Survival of patients and reconstructions, type of hinge, extensor mechanism reconstruction, MSTS function, and complications were analyzed., Results: Survival of patients with sarcomas was 68% and 62% at 5 and 10 years. Survival of megaprosthetic reconstructions was 82% and 78% at 5 and 10 years, without any difference between fixed and rotating hinge megaprostheses. MSTS function was significantly better in univariate and multivariate analysis for rotating compared to fixed hinge megaprostheses, without any difference between the types of extensor mechanism reconstructions. Most common complications were infection (12%), aseptic loosening (6%), and extensor mechanism rupture (3%)., Conclusions: Rotating hinge proximal tibia megaprosthetic reconstructions have better function compared to fixed hinge, regardless of the type of extensor mechanism reconstruction., (Copyright © 2012 Wiley Periodicals, Inc.)