Introduction: Sacrectomy is a primary intervention for high-grade sarcomas and aggressive benign bone tumors, combined anterior and posterior approach with spinopelvic fixation, contributing to prolonged surgical duration and increased wound complications. We aim to analyze perioperative and functional outcomes following sacrectomy using a posterior-only approach. Methods: Between 2018 and 2021, 26 patients underwent sacrectomy for primary malignant tumors. Perioperative outcomes were evaluated during hospitalization, with subsequent assessments conducted at 2 weeks, 6 weeks, 3 months, and every 6 months thereafter. Final functional outcomes were appraised following a minimum follow-up period of 24 months. Results: 6 patients underwent total sacrectomy, 14 had high partial sacrectomy preserving the S1 body, and 6 had sacrectomy below S2. The overall final Musculoskeletal Tumor Society (MSTS) score averaged 23 (range: 16 to 28), with 24 patients walking unaided, one using a cane, and one requiring a walker. Prophylactic colostomy was performed in 12 patients due to tumor proximity to the rectum. The average surgery duration was 236 minutes, with a blood loss of 2645 ml. Hemodynamic instability necessitating vasoactive drugs occurred in 8 patients postoperatively. Complications included intraoperative ureter injury 3.8% , sciatic nerve sacrifice 15%, rectum injury 3.8%, and vaginal injury 3.8%, wound complications required repeat debridement in 15% patients. No spinopelvic migration was detected on the final X-ray at 24 months. Conclusion: This pilot study suggests that, even without spinopelvic reconstruction, patients undergoing total sacrectomy with a posterior-only approach can achieve good MSTS scores and experience limited perioperative complications.

Introduction: En bloc resection of spinal tumors provides better local control and survival outcomes than intralesional resection. Safe margins during en bloc resection of primary spinal tumors with epidural involvement are required for improved outcomes. The present study describes a “Rotation-Reversion” technique that has been used for en bloc resection of primary tumors in the mobile spine with epidural involvement, as well as reporting the mid-term clinical outcomes in these patients. Methods: All patients with primary spine tumors who were treated with the Rotation-Reversion technique at our institution between 2015 and 2023 were evaluated retrospectively. Of the patients identified, those with both extraosseous soft tissue masses and epidural involvement were selected for case review. Clinical and radiological characteristics, pathologic findings, operative procedures, complications, and oncological and functional outcomes of these patients were reviewed. Results: 23 patients identified with primary spine tumor who underwent en bloc resection using the Rotation-Reversion technique between 2015 and 2023. Median follow-up time was 26.6 months, mean operation time was 679.6 min (range 220–1340 min) and mean blood loss was 5430.4 mL (range 1500–17700 mL), with 14 (60.9%) of the 23 patients experiencing perioperative complications. The negative margin rate was 91.3%, with five patient (21.7%) experiencing local recurrence. Conclusion: According to the mid-term clinical outcomes, the Rotation-Reversion technique is an effective procedure for the en bloc resection of primary spinal tumors to achieve more reliable and safe surgical margins.

Introduction. Due to prolonged cancer survival, bone metastases became an important issue in oncological patients, and the most frequent cause of death and disability. Bone is involved in a later stage of cancer disease and the spine is the most frequent site. The aim of the treatment of metastases remains control of disease and provide a satisfactory quality of life. 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. We reported our initial experience in treatment of spine metastasis with electrochemotherapy.
Methods. A systematic literature review was performed according to PRISMA guidelines. Operative protocol: treatment planning for electrode positioning and electrical pulse parameters was prepared for 6 needle electrodes. Open surgery with laminectomy was performed to introduce the eletrodes. The patients were treated according to the established Electrochemotherapy Protocol with Bleomycin
Results. Three patients with Mixofibrosarcoma, squamous cell carcinoma and leiomyosarcoma spinal metastases were treated. Neither serious electrochemotherapy-related adverse events, nor bleomycin toxicity were reported. Pain relief and neurological improvement have been observed in all cases.
Conclusions. According to literature review and our experience, ECT enhances the toxicity of bleomycin administrated systematically changing the permeability of membrane cells. This effect is possible due to modify membrane cells polarization induced by electric pulses carried near to the tumor site with several techniques. ECT does not modify overall survival of oncologic patients but is an alternative option of treatment of spine metastases.

Diagnosis and treatment for myofibroblastic sarcoma of bone are challenge because of its rareness and benign-like appearance. Here, we aimed to review the clinical features, evaluate the clinical outcome and explore gene alterations of this distinct tumor. 22 cases between 2009 and 2020 were included in our study. The average age was 33.9 years old. Tumors’ location varies, including 12 in femur, 5 in humerus, 3 in tibia, and 2 in pelvis. Seventeen patients were admitted to our hospital initially (Group 1), and five patients were transferred from other hospitals because of misdiagnosis and recurrence (Group 2). There were 12 patients with IB disease and 10 patients with IIB disease according to Enneking stage system. Two patients were received shoulder disarticulation. The other 20 patients were received local tumor en-block resection following different limb sparing reconstructions. Next generation sequencing was used to detect gene alterations of these tumors. All patients were followed-up with an average of 5 years. Five patients died because of multiple distant metastases and two patients got local tumor recurrence. The one-year and three-year survival were 84.1% and 70.7%, respectively. Overall survival of Group 2 patients was significant worse than that of Group 1(P<0.05). Rb missense mutation, KIT missense mutation and amplification were found. This disease can be easily misdiagnosed to benign bone tumor such as fibrous dysplasia. Recurrence of this tumor leads to a higher malignancy with multiple distant metastatic potential. Some targetable gene alterations may contribute to developing novel therapy for patients with this disease.

Giant cell tumors (GCTs) are managed surgically with the goal of sparing of the articular surface. These methods include polymethylmethacrylate (PMMA) cementation, and metal fixation with or without subchondral bone grafting, among others. Due to the difference in elastic modulus between PMMA and bone, there may be an increased risk of fracture or osteoarthritis in patients treated with PMMA. We sought to answer the following: Will the use of subchondral bone grafting affect rates of local recurrence, arthritis, and fracture? Medical records of patients treated for GCT from 1996-2022 at single institution were retrospectively reviewed. The patients were divided into two cohorts: PMMA only and bone graft. Information on adjuvant treatment, additional fixation, follow-up, presence of a fracture, arthritis, and local recurrence was obtained. The Musculoskeletal Tumor Society score percent was calculated. Fifty-two patients were analyzed.The mean age was 33 years with a mean follow-up of 91 months. Patients treated with PMMA alone had a 9x greater risk of arthritis and a 5x greater risk of fracture. The risk of recurrence following intervention was not statistically different. Functional analysis utilizing MSTS scores demonstrated an average of 92% with no statistically significant differences between the cohorts. The use of bone graft at the subchondral region in conjunction with PMMA was associated with reduced risk of later fracture or development of osteoarthritis at follow-up. This technique may delay or decreased the need for later revision surgery due to non-oncologic complications. No difference was seen in risk of local recurrence between groups.

Giant cell tumor of bone (GCTB) is a locally aggressive and rarely metastasizing neoplasm which is mainly treated with surgery. The main issue with the selection of the most appropriate surgical procedure is its high recurrence rate, possible malignant transformation and location near the joints which may lead to significant functional impairment. In this retrospective study, we analyze data from patients with GCTB who were treated in the period 2000-2020 at the University Clinic for Orthopedic Surgery- Skopje. This institution is a tertiary care center and referral center for bone tumors in North Macedonia. The following data of each patient were reviewed: age, sex, location of tumor, type of operative procedure, follow up and recurrence. In the study period, total of 80 patients were treated: 30 males (37,5%), 50 females (62,5%), age: 6-79 (median 35 years). There were total of 108 surgical procedures performed on 80 patients with GCTB. Follow-up 36-144 months (average 84 months), 2 patients lost during follow-up for non-related reasons, 13 patients (16,25%) with 17 local recurrences (21,25%). After the initial surgical treatment, 23 patients required a second surgery. Group of 5 patients were treated with additional surgery. There was no significant association between age, sex, location of GCTB and type of operative procedure with local recurrence. There was a statistically significant association between Campanacci staging of tumor and local recurrence. We hope that our study provides useful insights into the management and prognosis of this challenging tumor.

Introduction. Phosphaturic mesenchymal tumor causes severe hypophosphatemic osteomalacia poorly corrected by pharmacotherapy. Extreme rarity of the disease, latent course, and difficulties in diagnostic process leads to delayed diagnosis, severe deformities and fractures. Often observed infiltrative growth of the tumor creates high tendency of recurrence.

Aim. To carry out diagnostics and radical surgical treatment of patients with phosphaturic mesenchymal tumor.

Patients and Methods. 17 patients presented with severe hypophosphatemia, high serum alkaline phosphate level, osteopenia, fractures and bone deformities (8 females, 9 male, aged 32-65 years). Duration of clinical manifestations ranged 2-13 years. SPECT-CT with 99mTc-tectrotide or 68Ga-DOTATATE-PET/CT revealed tumors located in soft tissues in 10 cases, and in bones in 7 cases. MRI clarified its relation to anatomical structures. Follow-up ranged 1-5 years.

Results. In 16 cases en-block resection was performed, in 1 case excochleation was carried out. Twice there was R2 resection with removal of residual tumor with surrounding tissue, in both cases we observed no recurrence, all other cases were R0. In 2 cases morphological study showed absence of tumor, these patients developed metastases. Patient who went through excochleation had a local recurrence within 5 years. Normal serum phosphate level was noticed on the 5th day after surgery except for 2 with metastases, alkaline phosphatase – 2-3 months later. In 6 months radiographs showed significant increase in bone density, restoration of normal bone architectonics and consolidation of fractures.

Conclusion. Application of advanced imaging modalities and radical surgery provides good results in treatment of patients with phosphaturic mesenchymal tumor.

Objective:
To observe the similarities and differences in clinical manifestations, surgical treatment, and prognosis of OFD, OFD-AD, and AD.
Methods:
A retrospective review was conducted on cases of OFD, OFD-AD, and AD diagnosed in our unit from 2015 to 2022.Imaging and pathology of these three diseases were summarized, as well as the impact of different treatment methods on the prognosis of these three diseases.
Results:
A total of 41 patients were retrospectively included in this study, including 16 cases of OFD(mean age of 10.5 years), 19 cases of OFD-AD( mean age 5.3 years), and 6 cases of AD (mean age 29 years). 30 patients underwent intralesional resection (R2) surgery, and 6 patients underwent complete resection (R0-R1), 5 cases did not accept surgery. After a follow-up of at least 24 months (24-90 months, median 56 months), tumor recurrence or progression occurred in 11 patients (2/16, 12.5% in OFD; 8/19, 42% in OFD-AD; 1/6, 17% in AD). One patient had simultaneous diagnosis of OFD-AD (fibula) and AD (tibia), one patient had OFD-AD recurrence diagnosed as OFD 4 years after surgery, and one patient had OFD recurrence diagnosed as OFD-AD. No patient developed pulmonary metastasis.
Conclusion:
OFD, OFD-AD, and AD share similarities in clinical and imaging manifestations, and show an evolutionary relationship as different stages of the same type of lesion in pathological behavior. They have different peak ages of onset and vary at recurrence risks, therefore, accurate diagnosis and selection of appropriate treatment are crucial for these diseases.

Following limb salvage surgery at the knee in children, implantation of stemmed fixation opposite the resected bone can be performed via cementation with preservation of the open physis. Cementation technique can be performed either coating only the surface of the implant (“buttered”) or cementing the entire stem. We sought to analyze if growth was disturbed at the site of cemented stem fixation and if type of cementation technique altered growth differently. Patients treated with expandable endoprostheses at the knee following primary bone sarcoma at two tertiary centers were reviewed. Patients were followed from 2003-2024 and included if they underwent surveillance for limb length discrepancy (LLD) until skeletal maturity. Fourteen patients (8 males; 6 females) underwent resection of Osteosarcoma (13) or Ewing’s Sarcoma (1) at the distal femur (11) or proximal tibia (3). Mean age was 9.7±2.0 years. Mean follow-up was 122.2±62.9 months. Mean overall LLD at latest follow-up was 1.7±0.9cm. Two (14%) patients had growth disturbance > 2cm at the bone of stem fixation compared to contralateral. Mean growth of the stemmed bone versus contralateral bone was 4.0±1.5 vs 5.2±1.8cm; p=0.119. The impact of “buttered” (8) versus fully cemented (6) implants on LLD was comparable (0.4±0.1cm vs 0.6±0.6cm; p=0.36). Cemented endoprosthesis stems through an open physis disturbed growth incompletely at the proximal tibia and distal femur. Fourteen percent of patients had growth disturbance > 2cm at sites of fixation. Further larger studies are worthwhile to assess estimated growth disturbance more accurately in these fixation techniques.

The prognosis of patients after local recurrence of primary chondrosarcoma is heterogenous in the literature. The rates of local recurrence vary across grade, location, and surgical margins. We sought to answer: What factors influence local recurrence and prognosis in primary chondrosarcoma? A thorough literature review was conducted using the PubMed database. Included were peer-reviewed studies with at least 10 patients, that reported on local recurrence in the pelvis or long bones. Two independent reviewers screened the studies. Information was collected on tumor characteristics, surgical margins, and survival after local recurrence. Effect size was calculated via random effects model with residual maximum followed by a meta-analysis of proportions. 37 cases with 2,273 patients who received treatment for primary chondrosarcoma were analyzed. The overall summary proportion for local recurrence was 20.86 [95% CI 14.28 – 29.43]. Meta-regression indicated a positive association between presence of metastasis (p < 0.001) and grade 3 (p 0.012) lesions. Meta-regression revealed negative association between grade 1 lesions (p < 0.001), extremity lesions (p 0.002). Overall summary proportion of patients with local recurrence and alive with disease was 12.98 [95% CI 9.36 - 17.72] while those who died of disease was 8.95 [95% CI 5.67 - 13.85]. Metastasis and higher grade at diagnosis of chondrosarcoma were associated with local recurrence while extremity lesions were associated with lower local recurrence.

Surgical treatment of malignant tumors affecting the flat bones of the chest has always posed numerous challenges for surgeons. While modern imaging techniques, such as CT and MRI, facilitate precise resection planning based on oncological criteria, determining the most suitable approach for reconstructing the resulting defect remains a complex issue. A primary challenge arises from the chest's high mobility and flexibility, along with the need for multiple anchoring points for implant placement to ensure comprehensive and natural chest support. Insufficient support points often lead to implant instability, underscoring the ongoing need to investigate the optimal type for securing rib fragments post-resection. Malignant tumors originating in the sternum as a primary site are exceptionally rare in children, with limited descriptions of corresponding surgical interventions, prompting the development of a novel implant model. The developed prosthesis, tailored based on CT scan findings, not only ensures effective chest stabilization after extensive resection but also preserves mobility at the sternoclavicular joint. A unique experience of the replacement of the sternum handle in 4 patients demonstrates high efficiency in the long term compared to rigid constructs. The study outlines the enduring outcomes of using a prosthesis with a customized form, integrating titanium 3D printing technology and polymer compounds. The application of hybrid fixation methods has successfully delivered strong fixation of the sternum with a physiological amplitude of movements in the upper extremities.

Introduction: Tumors of the shoulder girdle and other flat bones have shown worse prognosis than tumors of the extremities. Limb salvage is the treatment of choice in approximately 95% of shoulder girdle tumors in current day practice. Reconstruction of shoulder girdle tumors by various means have not shown to provide any better function than scapulectomy alone in previous studies. Methodology:14 patients with benign and malignant tumors of shoulder girdle were included in this study. All patients underwent either a partial or total scapulectomy in a single institution. These patients were reviewed in the follow up period for functional outcomes. All patients were operated in the study period between 2017 and 2022. Functional outcomes were measured in terms of range of motion(adduction, abduction, flexion,extension,internal and external rotation) . The Musculoskeletal Tumor Society Score(MSTS) and DASH scores were also done to assess patient reported outcomes.Results: The mean age was 23.6(4-85). 5 patients had a benign tumor of the shoulder girdle and 9 patients had a malignant tumor and underwent scapulectomy.6 patients had a partial scapulectomy and 8 patients underwent a total scapulectomy. There was a significant difference in movements and patient reported outcome measures (MSTS and DASH scores) (p<0.005) between the partial and total scapulectomy groups Conclusion:Scapulectomy(partial/total) is a good treatment option for tumors of the shoulder girdle and avoids the need for forequarter amputation which has a significantly higher morbidity. Patients with partial scapulectomy had significantly better range of motion and functional outcomes compared to total scapulectomy

Introduction: Osteosarcoma is one of the most common primary malignant sarcomas of musculo-skeletal system, with bimodal age distribution. This study aims to investigate the possible role of microRNAs in the development of chemoresistance for their potential diagnostic and therapeutic applications. Study Design: Tumor tissue were taken from 13 cases of Osteosarcoma at time of diagnostic biopsy and tumor resection, after course of three cycles of neoadjuvant chemotherapy comprising of two drugs i.e., Doxorubicin and Cisplatin. Tumor tissue were stored in RNAlater solution at -80°C which were later processed and analysed for targeted MicroRNAs by RT-qPCR and patients were divided into two groups of good and poor responders to chemotherapy based upon necrosis seen in tumor resection specimen on histopathological analysis. Results: Based upon histopathological analysis, patients were divided into good and poor responders. A difference in fold change between good and poor responders was seen such that miR-22-3p is increased in poor responders while miR-301a-3p and miR-584 are increased in good responders. On analysing the fold change between two groups of good and poor responders by Unpaired t-test for all 3 microRNAs individually, the results were not significant as p values were more than 0.05. Conclusion: Our study had unique study design where tumor tissues were retrieved in vivo, from the human body during the course of NACT and then tested for microRNA expression. Relative expression of mir-584 revealed upregulation of miR-584 in good responders as compared to poor responders (fold change 5.9813) which is in concurrence with previous literature.