Classic Kaposi sarcoma: Diagnostics, treatment modalities, and genetic implications - A review of the literature.

BACKGROUND AND PURPOSE: Classic Kaposi sarcoma (CKS) is a rare vascular disease mainly found in populations of Mediterranean origin. The pathogenesis involves Human Herpes Virus 8 (HHV8) and genetic mutations such as SNP309 in the MDM2 gene. The recently discovered BPTF mutation in cells of CKS patients demonstrated higher latency-associated nuclear antigen (LANA) staining and altered vital transcriptomics, implicating a potential role in tumorigenesis. This review explores the genetic underpinnings and treatments for CKS. MATERIAL AND METHODS: A comprehensive literature search was conducted from 2004 to 2024, yielding 70 relevant papers. Ongoing clinical trials investigating novel treatments such as talimogene and abemaciclib were included in the search and presented in the results. RESULTS: Clinical diagnosis and treatment can be challenging as the number of studies on CKS and treatment modalities is limited. Treatment strategies vary by disease stage, with local therapies like surgical intervention and radiation therapy recommended for early stages, while systemic therapies are considered in cases of systemic disease. INTERPRETATION: While advancements in CKS treatment offer hope, further studies on immunotherapy are warranted to broaden the therapeutic options, such as anti-bromodomain or BPTF-targeted therapy.

Is There a Strength Deficit of the Quadriceps Femoris Muscle in Patients Treated Conservatively or Surgically after Primary or Recurrent Patellar Dislocations? A Systematic Review and Meta-Analysis.

Background: Patellar dislocation is a knee injury affecting generally young, active individuals, damaging joint ligaments and structures, and impacting sports activity and quality of life. Objective: This review aimed to evaluate the role of the quadriceps femoris muscle in knee extension and to consider whether extensor strength deficits are present in patients who have suffered from a primary or recurrent patellar dislocation and have been treated surgically or conservatively. Methods: This systematic literature review with meta-analysis was performed following the PRISMA Statement criteria. The search engines consulted to select studies were MEDLINE/PubMed, Scopus, and Web of Science/ISI. The JBI Critical Appraisal Checklist tools were applied for the quality assessment based on the specific study design. The outcomes were measurements of the knee extension force of the quadriceps femoris muscle, which were objectively quantifiable with an isokinetic or mobile dynamometer. Results: Of the 891 articles initially identified through the databases, 10 studies with a total of 370 patients were included in the analysis. The results indicated a strength deficit of the quadriceps in patients who had undergone a patellar dislocation, in comparison with the control group, when examining the uninvolved limb or in comparison with the pre-operative values. The overall effect size was large, with a value of -0.99. Conclusions: Our review concluded that after a primary or recurrent patellar dislocation, strength deficits of the quadriceps femoris muscle in the knee extension of the affected limb are frequently observed in surgically or conservatively treated patients. This deficit may persist even after a protracted follow-up of up to three years after injury.

Minimally Invasive Distal Metatarsal Diaphyseal Osteotomy (MIS-DMDO) for the Prevention and Treatment of Chronic Plantar Diabetic Foot Ulcers.

BACKGROUND: Diabetic foot is one of the major complications of diabetes, affecting 15% of patients with diabetes. This study aims to evaluate and compare the clinical and radiographic outcomes of patients with diabetes affected by forefoot plantar preulcerative or ulcerative lesions who have undergone minimally invasive distal metatarsal diaphyseal osteotomy (MIS-DMDO) to assess its efficacy in the prevention and treatment of chronic plantar diabetic foot ulcers (CPDFUs). METHODS: The study included 60 patients, 38 with preulcers and 22 with ulcers, with at least 2 years of clinical and radiologic follow-up. Clinical outcomes were assessed using the European Foot and Ankle Society (EFAS) score, the Foot Function Index (FFI), and the Manchester-Oxford Foot Questionnaire (MOXFQ). The radiographic evaluation was performed according to the Maestro criteria. RESULTS: Both groups improved in clinical and radiologic outcomes when comparing baseline measurements to those at the final follow-up. There were no statistical differences between preulcer and ulcer groups in terms of both clinical and radiologic outcomes, with the only exception being FFI, which was lower in the preulcerative group. In multivariate analysis, gender and glycated hemoglobin (HbA1c) were predictors of better outcomes. Specifically, FFI and MOXFQ (P < .05) exhibited larger improvements in females, while Maestro 1 and 2 were better in patients with lower HbA1c (P < .05). All patients were considered healed at the final follow-up. CONCLUSION: Carefully performed minimally invasive distal metatarsal diaphyseal osteotomy can be an effective approach to the care of impending or chronically present plantar diabetic foot ulcers.

Improvement of Motor Task Performance: Effects of Verbal Encouragement and Music-Key Results from a Randomized Crossover Study with Electromyographic Data.

External motivational stimuli have been shown to improve athletic performance. However, the neurophysiological mechanisms underlying this improvement remain poorly understood. This randomized crossover study investigated the effects of music and verbal encouragement on measures of muscle excitation and myoelectric manifestations of fatigue in the biceps brachii and brachioradialis muscles during an endurance task. Fifteen untrained (mean age 29.57 ± 2.77 years) and 13 trained individuals (mean age 32.92 ± 2.90 years) were included. The endurance task, performed to exhaustion, consisted of keeping the dominant arm flexed to 90 degrees while holding a dumbbell loaded to 80% of 1RM with a supine grip in three randomized conditions: standard, with self-selected music, and with verbal encouragement. The untrained subjects showed an increase in task duration of 15.26% (p < 0.003) with music and 15.85% (p < 0.002) with verbal encouragement compared to the condition without external stimuli. There were no significant differences in the myoelectric manifestations of fatigue between the different conditions. Regarding the muscle excitation metrics, although the mean amplitude, peak value, and area under the curve remained unchanged across conditions, a significant reduction in the trend coefficient, indicating motor unit recruitment over time, was observed with both music (biceps brachii: -10.39%, p < 0.001; brachioradialis: -9.40%, p < 0.001) and verbal encouragement (biceps brachii: -7.61%, p < 0.001; brachioradialis: -6.51%, p < 0.001) compared to the standard condition. For the trained participants, no significant differences were observed between conditions in terms of task duration and outcome measures related to muscle excitation and myoelectric manifestations of fatigue, suggesting the possible presence of a ceiling effect on motivation. These results highlight the important role of external motivational stimuli, such as music and verbal encouragement, in improving task performance in untrained subjects, probably through more effective and efficient recruitment of motor units.

Is still effective massive allograft reconstruction in parosteal osteosarcoma of the distal femur? Review of the literature and advantages of newer technologies.

PURPOSE: Parosteal Osteosarcoma is a well-differentiated, low-grade bone sarcoma. It most commonly occurs in the third decade of life, usually in the distal femur. This study aims to perform a literature review about the types of reconstructions reported and to analyze the results of an updated technique of resection using custom-made 3D-printed cutting guides. METHODS: We perform a systematic literature review about parosteal osteosarcoma, evaluating treatments, margins, local recurrence, complications, and functional results when available. We also report a case treated in our Center with a revisited technique introducing custom-made 3D-printed cutting guides. RESULTS: We analyzed 12 studies with a total of 151 patients. The distal femur was the most frequently reported site (81.5%). After distal femur resection, reconstruction was performed with graft in most cases (48%), followed by prosthetic reconstruction (40%). Margins were wide in 85.5% of cases. The total incidence of local recurrence was 11%. Functional results were excellent in all cases, with a mean MSTS score of 86%. In our case, with the help of the jigs, the surgical technique was relatively easy, graft fusion excellent and fast, margins wide, and functional results excellent. CONCLUSIONS: In the literature, the most commonly used type of reconstruction after resection is biological with graft. Indeed, despite the increasing number of prosthetic reconstructions, the historical diaphysometaphyseal hemiresection and graft is still indicated in parosteal osteosarcoma of the distal femur. New technologies, such as the jigs we used, allow significant advantages during the procedure: reduce the resection and graft preparation time, allow a better match between components, and help to obtain safer margins, sparing as much bone as possible.

Nursing Leadership in a Post-Pandemic Elective Orthopaedic Theatre Department: A Detailed Thematic Analysis of an Open-Ended Qualitative Survey.

BACKGROUND: The COVID-19 pandemic has impacted nursing theatre staff, departmental activity, and delivery of services to patients. This work-based project aimed to investigate the challenges of nursing leadership in an elective orthopaedic department at current times. METHODS: The study collected qualitative data exploring theatre staff's expectations from leadership, offering insight on how the pandemic has influenced the way of working and exploring how the future in this unit may look. The answers from 20 practitioners to an anonymised open-ended survey were examined using thematic analysis. RESULTS: The participants described a leader as a good communicator who focuses on empowering others and supporting the team, identified by the majority as a senior team member. From the findings, three topics were identified: immediate changes, delayed changes, and pre-existing conditions. The answers painted a reality that is complex and multifaceted, where numerous variables play a part in the physical and mental health of each candidate, impacting their performance as well as their work/life balance. Overall, the strongest subjects recurring in the findings were the need for nursing leadership to focus on supporting staff with training opportunities, to actively plan for a reduction in staffing shortages, and to be constantly mindful of staff well-being. CONCLUSIONS: This study pointed out that the need for constant communication with their staff, building honest relationships, and being a reliable leader, focused on empowering others and supporting the team were important factors for the nursing management during the COVID-19 pandemic and post-COVID-19 era.

Reconstructive surgery after distal fibular resection due to bone tumors: a technical report on surgical strategies and results from the PROSPERO international register of systematic reviews.

Purpose: Primary bone tumors of the fibula are rare. Distal fibular resection has a significant impact on ankle biomechanics and gait, possibly leading to complications such as ankle instability, valgus deformity, and degenerative changes. Question: Is there a need for reconstructive surgery after distal fibular resection, and what reconstructive procedures are available? Materials and methods: The review is registered with the PROSPERO International Register of Systematic Reviews. Inclusion criteria consisted of all levels of evidence, human studies, patients of all ages and genders, publication in English, and resection of the distal portion of the fibula due to tumor pathology. The reviewers defined four different categories of interest by method of treatment. Additional articles of interest during full-text review were also added. Results: The initial search resulted with a total of 2958 records. After screening, a total of 50 articles were included in the study. Articles were divided into 'No reconstruction', 'Soft tissue reconstruction', 'Bone and soft tissue reconstruction', and 'Arthrodesis, arthroplasty or other reconstruction options' groups. Conclusion: Limb salvage surgery should be followed by reconstruction in order to avoid complications. Soft tissue reconstructions should always be considered to stabilize the joint after fibular resection. Bone reconstruction with reversed vascularized fibula is the preferred technique in young patients and in cases of bone defects more than 3 cm, while arthrodesis should be considered in adult patients. Whenever possible for oncologic reason, if a residual peroneal malleolus could be preserved, we prefer augmentation with a sliding ipsilateral fibular graft.

Investigating and Practicing Orthopedics at the Intersection of Sex and Gender: Understanding the Physiological Basis, Pathology, and Treatment Response of Orthopedic Conditions by Adopting a Gender Lens: A Narrative Overview.

In the biomedical field, the differentiation between sex and gender is crucial for enhancing the understanding of human health and personalizing medical treatments, particularly within the domain of orthopedics. This distinction, often overlooked or misunderstood, is vital for dissecting and treating musculoskeletal conditions effectively. This review delves into the sex- and gender-specific physiology of bones, cartilage, ligaments, and tendons, highlighting how hormonal differences impact the musculoskeletal system's structure and function, and exploring the physiopathology of orthopedic conditions from an epidemiological, molecular, and clinical perspective, shedding light on the discrepancies in disease manifestation across sexes. Examples such as the higher rates of deformities (adolescent idiopathic and adult degenerative scoliosis and hallux valgus) in females and osteoporosis in postmenopausal women illustrate the critical role of sex and gender in orthopedic health. Additionally, the review addresses the morbidity-mortality paradox, where women, despite appearing less healthy on frailty indexes, show lower mortality rates, highlighting the complex interplay between biological and social determinants of health. Injuries and chronic orthopedic conditions such osteoarthritis exhibit gender- and sex-specific prevalence and progression patterns, necessitating a nuanced approach to treatment that considers these differences to optimize outcomes. Moreover, the review underscores the importance of recognizing the unique needs of sexual minority and gender-diverse individuals in orthopedic care, emphasizing the impact of gender-affirming hormone therapy on aspects like bone health and perioperative risks. To foster advancements in sex- and gender-specific orthopedics, we advocate for the strategic disaggregation of data by sex and gender and the inclusion of "Sexual Orientation and Gender Identity" (SOGI) data in research and clinical practice. Such measures can enrich clinical insights, ensure tailored patient care, and promote inclusivity within orthopedic treatments, ultimately enhancing the precision and effectiveness of care for diverse patient populations. Integrating sex and gender considerations into orthopedic research and practice is paramount for addressing the complex and varied needs of patients. By embracing this comprehensive approach, orthopedic medicine can move towards more personalized, effective, and inclusive treatment strategies, thereby improving patient outcomes and advancing the field.

Controversies in orthopaedic oncology.

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.

Electrochemotherapy in Spine Metastases: A Case Series Focused on Technical Aspects, Surgical Strategies and Results.

Metastases are complications of primary tumors due to prolonged cancer survival and have become an important issue for oncological patients and the most frequent cause of death and disability. Bone metastases occur at a later stage of cancer disease, and the spine is the most frequent site. To date, the aim of the treatment of metastases remains to be the control of disease and provide a satisfactory quality of life. The decision making of treatment is influenced by several factors such as the status of the primary disease, the number of metastases, site involvement, and the performance status of the patients. For this reason, the treatment of metastases is challenging and undergoes constant development. Therefore, alternative techniques with respect to surgery, which is the first option but not always practicable, and radiochemotherapy are attractive. Lately, electrochemotherapy has emerged as an innovative method for treating various primary and metastatic solid tumors, showing promising outcomes in terms of inducing tumor tissue necrosis and alleviating symptoms. This technique uses electric pulses to increase the uptake of chemotherapy by tumor cells. Despite the initial enthusiasm and good results in the treatment of bone tumors, relatively few papers have described its use in spine metastases. Therefore, we conducted a systemic review of this intriguing topic while also reporting our experience in the use of electrochemotherapy for the treatment of spine metastases.

Modern treatment of unicameral and aneurysmatic bone cysts.

The best treatment of unicameral bone cyst and aneurismatic bone cyst (ABC) is debated in the literature. For simple bone cysts, multiple treatments were proposed from observation only to open curettage. The historical treatment with intraosseous injection of methylprednisolone acetate into the bone cysts nowadays is reduced due to the morbidity of multiple injections and the risk of multiple pathologic fractures until the healing. Different types of treatments for ABC are reported, including surgery, percutaneous treatments, and medical treatments; however, there is currently no consensus on the best approach. The association of curettage, bone graft, and elastic stable intramedullary nail (ESIN) had a success rate of over 85%. Decompressing the cyst wall is more critical for increasing the healing rate than the type of graft used to fill the cavity. In ABC, sclerotherapy offers the advantages of lower invasiveness and morbidity, associated with better functional scores and faster return to full weight-bearing. Moreover, they can be used in challenging locations. Selective arterial embolization is a complex procedure and often requires association with other treatments. Further studies are needed to confirm the effectiveness of denosumab and its side effects on skeletally immature patients. Curettage with adjuvants and autogenous bone grafting still shows promising results and can be used in larger, aggressive defects or superficial lesions. For simple bone cysts, the combination of curettage, bone graft, and ESIN showed the best results. Sclerotherapy for ABC also shows promising results.

The Evaluation and Management of Lung Metastases in Patients with Giant Cell Tumors of Bone in the Denosumab Era.

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.

Ortho-oncoplastic surgery in foot and ankle: A narrative overview on reconstruction of soft-tissue defects after oncologic resections.

INTRODUCTION: Malignant tumors of the foot are rare, and treatment strategies are challenging considering the complex anatomy of this area. In recent years, dramatic advances in technology and collaborations between different specialties (such as orthopedic, oncology, radiology, plastic, and vascular surgery) significantly changed the approach to complex malignant tumors without resorting to limb removal. The combination of the strengths of both orthopedic surgery and plastic surgery constitutes the modern definition of "orthoplasty." The aim of this review article is to provide treatment strategies that are available for reconstruction of foot and ankle in limb-salvage surgery after tumor resection, with a specific focus on microsurgical techniques in plastic surgery. METHODS: We conducted a comprehensive search for relevant papers across PubMed, Scopus, Embase, and Web of Science. We included patient-based studies reporting on procedures for soft-tissue reconstruction with small and large soft tissue defects. Indications, pros and cons, and technique tips are discussed for each type of reconstructive technique. RESULTS: The search was done using literature of the past 30 years (from 1990 to date), resulting in about 725 articles describing over 2000 cases. Cutaneous flaps included lateral supramalleolar flap, medial plantar flap, reverse sural neurocutaneous island flap, medial leg flap, and lateral leg flap. Free flaps included anterolateral thigh flap, radial forearm flap, latissimus dorsi flap, gracilis muscle flap, lateral arm flap, and rectus abdominis flap. CONCLUSIONS: The orthoplastic approach in musculoskeletal oncology is a collaborative model of orthopedic and plastic surgeons working together, resulting in a higher rate of successful limb salvage in patients at risk for amputation. Protocols, biologic substitutes, and surgical techniques are largely improved in the last decades increasing the possibility of functional reconstruction. Microsurgical strategies represent the new frontiers in these demanding reconstructions.

Adaptive Shooting Disciplines: A Scoping Review of the Literature with Bibliometric Analysis.

Para-archery and para-shooting, two very popular adaptive shooting disciplines that have earned their place as major official events in the Paralympic Games, share some similarities, as well as distinctive features in terms of rules, physiological requirements, and equipment used. The International Paralympic Committee has a clear responsibility to ensure that all sports within its jurisdiction, including adaptive shooting, can achieve excellence in their respective fields. To achieve this, the conduct of well-designed studies and rigorous research is essential. Although some research has been conducted in this area, a comprehensive and systematic evaluation is still needed. Therefore, the present study aims to provide a thorough review and synthesis of existing research on adaptive shooting disciplines, identify strengths and gaps, and suggest future directions. Arksey and O'Malley's methodology is leveraged and enhanced with bibliometric and policy analyses to review literature on adaptive shooting. Databases like PubMed/MEDLINE, Scopus, Web of Science, OvidSP, and EMBASE were searched, focusing on studies in adaptive shooting disciplines and analysing these findings through a blend of thematic and statistical methods. Twenty-four studies totalling 483 para-athletes (299 para-shooters and 184 para-archers) are included in this scoping review, focusing on a range of aspects, including physiological responses (n = 9), research design and measurement methods for evidence-based classification (n = 6), biopsychosocial aspects (n = 3), development of new methods and technologies (n = 4), kinematic and biomechanical assessment (n = 1), and epidemiology of injuries (n = 1). Seven articles focused exclusively on para-archery, thirteen exclusively on para-shooting, and four on both para-archery and para-shooting. In conclusion, the available literature on adaptive shooting disciplines is still very limited, especially regarding para-archery compared to para-shooting. This highlights the need for further research in many key areas to ensure a better understanding of the different disciplines and to provide appropriate support for para-athletes. Future research in para-archery and para-shooting should focus on technological innovations, biomechanical studies, and psychological support to enhance athlete performance and accessibility. Addressing the imbalance between the two disciplines, along with injury prevention and global participation, can drive the sports towards greater inclusivity and equity for para-athletes worldwide.

When Is a Two-Stage Surgical Procedure Indicated in the Treatment of Pseudotumors of the Hip? A Retrospective Study of 21 Cases and a Review of the Literature.

(1) Background: A pseudotumor of the hip is a sterile, non-neoplastic soft tissue mass associated with total hip arthroplasties. Pseudotumors may mimic soft tissue tumors or infections, and thus a differential diagnosis is crucial, and biopsy is recommended. The purpose of this study was to compare the complications and functional results between one-stage and two-stage procedures. (2) Methods: We retrospectively analyzed 21 patients surgically treated at our institution with "pseudotumors" associated with hip prosthesis (8 male, 13 female with a mean age of 69 years). One-stage revision was performed in 10 cases and two-stage reversion in 10, with excision only in 1 case. Complications were classified as major and minor and functional results assessed using the Harris Hip Score (HHS). (3) Results: Five patients (24%) reported major complications. The survival rate for all complications was 75%. The overall survival rate was 95% at 5 years. The mean HHS ranged from 35 pre-op to 75 post-op, highlighting improved functional results in all cases. We recorded no differences in complications or functional outcomes between the one- and two-stage procedures. (4) Conclusions: In our experience, the two-stage surgical approach is preferable in cases with major bone defects and larger pseudotumor sizes. The use of custom-made 3D-printed prostheses is increasing and is a further reason to prefer two-stage revision.

Are IDH1 R132 Mutations Associated With Poor Prognosis in Patients With Chondrosarcoma of the Bone?

BACKGROUND: Because chondrosarcomas vary widely in their behavior, and because anticipating their behavior based on histology alone can be challenging, genetic markers represent an appealing area of inquiry that may help us refine our prognostic approaches. Isocitrate dehydrogenase (IDH) mutations are involved in the pathogenesis of a variety of neoplasms, and recently, IDH1/2 mutations have been found in the tissue of benign cartilage tumors as well as in conventional chondrosarcomas and highly aggressive dedifferentiated chondrosarcomas. However, their association with patient survival is still controversial. QUESTIONS/PURPOSES: (1) What proportion of patients with chondrosarcomas carry IDH mutations, and which IDH mutations can be found? (2) Are any specific IDH mutations associated with poorer overall survival, metastasis-free survival, or local recurrence-free survival? METHODS: Between April 2017 and December 2022, we treated 74 patients for atypical cartilaginous tumors or chondrosarcomas in a musculoskeletal tumor referral center. Patients were considered potentially eligible for the present study if the histologic diagnosis was confirmed by two expert soft tissue and bone pathologists following the current WHO classification, complete preoperative imaging and follow-up data were available, surgical excision was performed by sarcoma orthopaedic surgeons directed by a team leader, and the minimum follow-up was 2 years after surgical treatment unless the patient died. Data including sex, age, diagnosis, grade, type of operation, local recurrence, metastasis, and oncologic follow-up were recorded. Forty-one patients (55%) were eligible for the study. For each patient, DNA was extracted and quantified from paraffin-embedded sections of tumor tissue, and the mutational status of IDH1 (codons 105 and 132) and IDH2 (codons 140 and 172) genes was assessed. Of those, 56% (23 of 41) of patients had adequate DNA for analysis of IDH mutations: 10 male and 13 female patients, with a median age of 59 years (range 15 to 98 years). There were 22 conventional chondrosarcomas (8 atypical cartilaginous tumors, 11 Grade 2, and 3 Grade 3) and 1 dedifferentiated chondrosarcoma. Stage was IA in 3 patients, IB in 5, IIA in 1, IIB in 13, and III in 1, according to the Musculoskeletal Tumor Society classification. At a median follow-up of 3.5 years (range 4 months to 5.6 years), 14 patients were disease-free, 2 were alive with disease, and 7 died (3 within 2 years from surgery). Eight patients had metastases, and 7 developed local recurrence. We determined the proportion of patients who carried IDH mutations, and compared patients with and without those mutations in terms of overall survival, metastasis-free survival, and local recurrence-free survival using Kaplan-Meier curves. RESULTS: Six patients showed wild-type IDH genes, and 17 had IDH mutations (12 had IDH1 R132, 3 had IDH1 G105, and 2 had IDH2 R172). Overall survival at 2 years using the Kaplan-Meier estimator was lower in patients with an IDH mutation than in those with the wild-type gene (75% [95% confidence interval 50% to 99%] versus 100% [95% CI 100% to 100%]; p = 0.002). Two-year metastasis-free survival was also lower in patients with an IDH mutation than in those with the wild-type gene (33% [95% CI 7% to 60%] versus 100% [95% CI 100% to 100%]; p = 0.001), as was 2-year local recurrence-free survival (70% [95% CI 42% to 98%] versus 100% [95% CI 100% to 100%]; p = 0.02). CONCLUSION: We found that IDH1 R132 mutations were negatively associated with the prognosis of patients with bone chondrosarcomas. Nevertheless, more extensive studies (such as multicenter international studies) are needed and advisable to confirm our observations in this preliminary small series. Moreover, evaluating mutational status in fresh samples instead of in paraffin-embedded sections could help to increase the number of patients with adequate DNA for analysis. If our findings will be confirmed, the evaluation of IDH mutational status in biopsy samples or resection specimens could be considered when stratifying patients, highlighting those who may benefit from more aggressive treatment (such as adjuvant chemotherapy) or closer follow-up. LEVEL OF EVIDENCE: Level III, prognostic study.

Primary vascular tumors of bone: A comprehensive literature review on classification, diagnosis and treatment.

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.