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Introduction: The periacetabular bone defects caused by metastatic disease often necessitate acetabular reconstruction and various techniques have been employed with varying degrees of success. The purpose of this study was to evaluate the efficacy and safety of acetabular reconstruction in conjunction with adjuvant microwave ablation as a surgical intervention for patients with periacetabular metastases. Methods: Between January 2019 and September 2023, 17 consecutive patients with different tumor subtypes required surgical intervention for periacetabular metastases. The acetabular reconstruction was performed by utilizing an acetabular reconstructive cage and cement total hip arthroplasty with microwave ablation. A retrospective review was performed to assess pain relief, intraoperative details and postoperative complications. Functional status following procedures was determined by the 1993 Musculoskeletal Tumor Society (MSTS) score and the overall survival of patients was estimated by Kaplan-Meier analysis Results: In total, 8 males and 9 females were included with an average age of 48.6 years (range 34-66). Patients reported a significant improvement in the level of pain and the mean VAS score declined from 7.7 preoperatively to 2.2 postoperatively. Of the 17 patients, 16 could ambulate either independently (6 patients) or using a walking aid (10 patients) with a mean MSTS score of 18.9. The median follow‐up was 9.0 months. Nine patients were alive at the most recent follow-up with overall survival of 40.9% at 12 months and 30.7% at 36 months, respectively. Conclusion: In selected patients with periacetabular metastasis, the utilization of an acetabular cage and cement total hip arthroplasty presents a less invasive reconstruction technique. The incorporation of adjuvant microwave ablation has shown promise in providing long-lasting pain relief, reducing intraoperative bleeding, and improving local tumor control. However, further research and extended follow-up are necessary to establish the effectiveness of this procedure. [ABSTRACT FROM AUTHOR]

Background: Fibrodysplasia ossificans progressiva (FOP) is an extremely rare disease characterized by malformation of the bilateral great toes and progressive heterotopic ossification. The clinical features of FOP occur due to dysfunction of the bone morphogenetic protein (BMP) signaling pathway induced by the mutant activin A type I receptor/activin‐like kinase‐2 (ACVR1/ALK2) which contributes to the clinical features in FOP. Dysregulation of the BMP signaling pathway causes the development of osteochondroma. Poor awareness of the association between FOP and osteochondromas always results in misdiagnosis and unnecessary invasive operation. Case Presentation: In this study, we present a case of classical FOP involving osteochondroma. An 18‐year‐old male adolescent, born with deformity of bilateral big toes, complained multiple masses on his back for 1 year. The mass initially emerged with a tough texture and did not cause pain. It was misdiagnosed as an osteochondroma. After two surgeries, the masses became hard and spread around the entire back region. Meanwhile, extensive heterotopic ossification was observed around the back, neck, hip, knee, ribs, and mandible during follow‐up. Osteochondromas were observed around the bilateral knees. No abnormalities were observed in the laboratory blood test results. Whole exome sequencing revealed missense mutation of ACVR1/ALK2 (c.617G > A; p.R206H) in the patient and confirmed the diagnosis of FOP. Conclusion: In summary, classical FOP always behaves as a bilateral deformity of the big toes, as well as progressive ectopic ossification and osteochondromas in the distal femur and proximal tibia. An understanding of the association between osteochondromas and FOP aids in diagnosis and avoids unnecessary invasive management in patients. [ABSTRACT FROM AUTHOR]

Giant cell tumor of bone (GCTB) is a rare, benign, but locally aggressive bone tumor. It has a high tendency for local recurrence, which may increase the incidence of lung metastasis. Currently, an optimal treatment strategy has not been established because of the rarity of pulmonary metastatic GCTB. Denosumab is the preferred regimen for unresectable metastatic lesions; however, there are no alternative treatment options when patients are resistant to denosumab. Apatinib is a small-molecule tyrosine kinase inhibitor that selectively competes for the vascular endothelial growth factor receptor 2 (VEGFR-2) ATP binding site, and several studies have analyzed the effectiveness of apatinib in advanced or metastatic tumors. However, there is no report of apatinib as an anti-angiogenesis therapy for pulmonary metastatic GCTB to date. Here, we present a case of a 26-year-old female who was diagnosed with recurrent and pulmonary metastatic GCTB. Immunohistochemical (IHC) staining indicated that the tumor cells were positive for VEGFR-2. Denosumab was administered to control the metastases; nevertheless, disease progression was confirmed after four months of treatment. Given the IHC results and rapid disease progression, apatinib was added to the treatment strategy. After 42 months of treatment, the patient showed noticeable symptomatic improvement and considerable tumor shrinkage. [ABSTRACT FROM AUTHOR]

F-box proteins are essential components of the Skp-cullin-F-box complex (a type of E3 ubiquitin ligase), and participate in cell cycle and immune responses through the ubiquitin proteasome system. F-box protein 39 (FBXO39) belongs to the F-box family, which has been reported to be associated with cancer oncogenesis and progression. The present study aimed to investigate the role of FBXO39 in osteosarcoma (OS) cell proliferation and apoptosis in vitro. It was demonstrated that U-2OS cells exhibited high expression of FBXO39 compared with HOS and SaOS-2 osteosarcoma cells. Thus, knockdown of FBXO39 was performed using lentivirus-mediated short hairpin RNA (shRNA) transfection to validate the effect of FBXO39 in U-2OS cells. Western blotting and RT-qPCR analysis were used to confirm the efficiency of infection by analyzing the expression level of FBXO39. Using Celigo-based cell counting and MTT assays, it was demonstrated that FBXO39 knockdown significantly reduced the rate of cell proliferation compared with control. Caspase 3/7 activity assays and fluorescence-activated cell sorting confirmed the induction of apoptosis in U-2OS cells following FBXO39 knockdown. In conclusion, it was demonstrated that FBXO39 knockdown may significantly inhibit proliferation and promote apoptosis of U-2OS cells. Thus, FBXO39 may serve an important role in OS progression. [ABSTRACT FROM AUTHOR]

The distal radius is an extremely rare site for epithelioid hemangioendothelioma. Joint preservation to maintain a good wrist joint function is rarely reported. The present study described a case of joint preservation surgery in the distal radius with an uncemented 3D-printed endoprosthesis and evaluated the endoprosthesis design and short-term outcomes. A 14-year-old boy was diagnosed with epithelioid hemangioendothelioma in radius. Due to the extensive defect and the excessively short length of the residual distal radius after resection, a custom-made 3D-printed custom-made endoprosthesis was designed and fabricated to reconstruct the defect, with the preservation of the wrist joint. The patient had a favorable wrist function and no endoprosthesis-related complications were observed. The present study presented a case of en bloc tumor resection with joint preservation of the wrist and reconstruction using a 3D-printed endoprosthesis. Satisfactory postoperative function and low complication rates were found. [ABSTRACT FROM AUTHOR]

Introduction: Limb-salvage surgery has become the mainstream approaches for the treatment of sarcoma in the lower extremity. In cases where the sarcoma infiltrates the primary vessel, concurrent resection of the vessels and vascular reconstruction are required to ensure sufficient resection and preservation of limb function. The objective of this study is to assess the clinical outcomes of patients who underwent vascular reconstruction utilizing synthetic grafts for limb salvage, specifically in terms of postoperative complications and limb functional status., Methods: Between September 2016 and October 2021, 15 consecutive patients who underwent 15 arterial and 3 venous reconstruction procedures were included in this retrospective study. Incidence of postoperative morbidity, graft patency, rate of limb salvage, and overall survival of patients were analyzed., Results: The median follow-up was 12.5 months (range, 4.5-72.0). Graft thrombosis occurred in 5 patients (33.3%) and graft occlusion occurred in 3 patients (20.0%). The median overall survival was 28.0 months with the estimated 2-year and 5-year overall survival of 57.8% and 43.4% respectively. The 1-year and 2-year estimated patency rates of arterial reconstructions were 82.3% and 62.1%, respectively. None of the included patients with limb amputation were observed as a consequence of severe vascular complications, while two patients underwent amputation due to the repeat recurrence, resulting in a limb salvage rate of 86.7%., Conclusion: Our results show that the combination of vascular reconstruction and oncologic resection is a feasible option for preserving limbs in cases of musculoskeletal sarcoma with vessel involvement in the lower extremity. When vascular reconstruction surgery is performed, synthetic substitutes can be effectively used with low perioperative morbidity and an acceptable rate of limb salvage., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Zheng, Xu, Li, Weng, Yang, Wang and Zhang.)

Background: Patients who undergo massive femoral malignant tumor (MFMT) resection often exhibit shortened femoral metaphyseal juxta-articular segments. The use of a customized femoral endoprosthesis (CFE) with an intra-neck curved stem (INCS) has emerged as a viable reconstructive surgical strategy for these individuals. Relative to a cemented INCS, it remains unclear as to whether cementless INCS use is associated with improvements in functionality or reconstructive longevity. As such, the present study was conducted to compare functional outcomes, endoprosthetic survival, and endoprosthesis-related complication rates in patients undergoing cemented and cementless INCS implantation., Methods: A total of 24 patients undergoing lower limb salvage and reconstructive surgical procedures utilizing cemented or cementless INCS endoprostheses were retrospectively included. Patient-functional outcomes, endoprosthetic survival, and complication rates were compared as a function of age; diagnosis; the length of femoral resection; residual proximal femur length; Musculoskeletal Tumor Society (MSTS) scores; visual analog scale (VAS) scores; and the rates of implant breakage, periprosthetic infections, periprosthetic fractures, and aseptic loosening., Results: The mean follow-up was 56 months. Significant differences in the length of femoral resection (p<0.001) and residual proximal femur length were observed (p<0.001) between the cemented and cementless INCS groups. There were no differences in overall patient survival and aseptic loosening-associated endoprosthesis survival in the cemented and cementless groups. None of the included patients experienced periprosthetic fractures, infections, or implant breakage. Average respective MSTS and VAS scores did not differ between groups., Conclusion: For patients undergoing treatment for MFMTs, the use of a CFE with an INCS has emerged as a viable approach to hip-preserving reconstructive surgery. With appropriately designed individualized rehabilitative programs, good functional outcomes can be achieved for these endoprostheses, which are associated with low complication rates. Moreover, the selection between cemented or cementless INCS in the clinic should be made based on patient-specific factors, with cementless INCS implementation being preferable in younger patients with good-quality bone, the potential for long-term survival, and the osteotomy site near the lesser trochanter, whereas cemented INCS use should be favored for individuals who are older, have a shorter life expectancy, or have poor bone quality., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 You, Lu, Min, Luo, Zhang, Wang, Zheng, Zhou and Tu.)

Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma. In view of the indolent course throughout the prolonged natural history of EMC, it was considered as a low-grade soft-tissue sarcoma. However, recent studies have revealed a high recurrence and metastatic potential in EMC, and the invasiveness of EMC may progress during the protracted clinical course. The mechanism for this aggressive transformation remains unknown. Here, we present a rare case of EMC with aggressive behavior. This case was confirmed via pathology and NR4A3 fluorescent in situ hybridization. To verify the genetic characteristics of this rare case, a total gene sequencing analyses was performed in the recurrent and metastatic lesions. Intriguingly, different gene mutations were determined in the recurrent and metastatic lesions, which implied the genetic heterogeneity among the different lesions might be related to the aggressiveness of EMC. Furthermore, we discuss a few potential agents against the mutated genes in this case, which may provide novel insights regarding the targeted therapy of EMC., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 He, Wang, Zou, Zheng, Luo, Zhou and Tu.)

Introduction: Few patients presented with a distal tibial tumor that only invaded a small area of bone in the medial malleolus. There have been no previous cases in which only the medial or lateral malleolus was removed and reconstruction was complete. This article describes our attempt to reconstruct the medial malleolus (1/4 of the ankle joint) after resection of a distal tibial tumor with an uncemented three-dimensional (3D)-printed prosthesis., Case Description: A 39-year-old man presented with a lump in the right medial malleolus, and biopsy results suggested fibrosarcoma. To preserve the patient's normal bone and function, we only removed the medial malleolus and reconstructed the ankle joint using a personalized 3D-printed prosthesis. The patient had no complications other than necrosis of the skin flap that covered the wound. The patient recovered well after undergoing an additional skin flap transfer. Follow-up at 7 months and again at 3 years after surgery showed good ankle function and stability, with no pain or complications., Conclusion: The 3D-printed partial ankle prosthesis had a good matching degree, strength, and osseointegration ability, but also had a few complications. The patient achieved satisfactory ankle function and stability. However, a longer follow-up period is needed, and more research is required to confirm the efficacy of the prosthesis., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Wang, Luo, Zhang, Wang, Zheng, Tu and Zhou.)

Background: Hemiarthroplasty is widely used for proximal femoral reconstruction after tumor resection. However, complications of hemiarthroplasty include infection, hip dislocation, and acetabular wear. This study aimed to: (1) evaluate the reliability and validity of a customized cementless intercalary endoprosthesis (CCIE) with an intra-neck curved stem (INCS) to reconstruct femoral diaphyseal defects with an ultrashort proximal femur (UPF); (2) assess the lower extremity function after reconstruction with this endoprosthesis; and (3) identify the postoperative complications associated with the use of this endoprosthesis. Methods: Between October 2015 and May 2019, 13 patients underwent reconstruction with a CCIE with an INCS. The distance from the center of the femoral head to the midline of the body and the apex of the acetabulum was measured preoperatively. Additionally, the distance from the tip of the INCS to the midline of the body and the apex of the acetabulum was measured postoperatively. The femoral neck-shaft angle was also measured pre- and postoperatively. After an average follow-up duration of 46 months, the radiological outcomes of the CCIE with an INCS were analyzed. Function was evaluated with the Musculoskeletal Tumor Society (MSTS) score. Pain was measured using a paper visual analog scale (VAS) pre- and postoperatively, and complications were recorded. Results: Compared with our preoperative design, we found no significant difference in the postoperative distance from the tip of the INCS to the body midline ( p = 0.187) and the apex of the acetabulum ( p = 0.159), or in the postoperative femoral neck-shaft angle ( p = 0.793). Thus, the INCS positions were deemed accurate. The average MSTS score was 26 (range: 24-28), and the VAS score was significantly decreased postoperatively compared with preoperatively ( p < 0.0001 ). No patients developed aseptic loosening, infection, periprosthetic fracture, or prosthetic fracture as of the last follow-up. Conclusion: The CCIE with an INCS was a valid and reliable method for reconstructing femoral diaphyseal defects with a UPF following malignant tumor resection. Postoperative lower extremity function was acceptable, with an appropriate individualized rehabilitation program, and the incidence of complications was low., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 You, Lu, Min, Zhang, Wang, Wang, Zheng, Zhou and Tu.)

Background: Ewing sarcoma is the second most common malignant bone tumor in children, but it rarely originates from extra-skeletal sites. The commonly involved sites of soft tissue include paravertebral spaces, lower extremities, the pelvis, head, and neck, while primary extra-skeletal Ewing sarcoma (EES) located in the genitals is extremely rare. Case Presentation: We report a young patient who presented to our hospital with a painful erection of the penis and limited motion of the left hip. Magnetic resonance imaging showed a hyperintense mass with invasion of adjacent tissue in the penis and a heterogeneously high signal lesion in the left proximal femur. 18 F-fluorodeoxyglucose positron-emission tomography detected widespread metastatic lesions in the bilateral lung and multiple skeletons. An incisional biopsy of the penis was performed; the histopathological findings and EWS gene translocation identified by molecular analysis confirmed the diagnosis of Ewing sarcoma. Subsequently, the punch-biopsy specimen from the left femur showed undifferentiated small round cells, a finding consistent with the microscopic presence of Ewing sarcoma metastasis. However, after the first course of multiagent chemotherapy, the penile mass did not obtain stabilization but instead grew progressively with surface ulceration and multidrug resistant bacteria infection. Despite receiving antibiotics and maximal supportive therapy, the patient died from sepsis and lung metastasis complications in the intensive care unit 2 months later. Conclusion: This case indicates that although EES as a subtype of Ewing sarcoma is rare, it can occur virtually in any soft tissue site, even in the genitals. Therefore, clinicians need to distinguish this entity from other soft tissue sarcomas with rapid progression since early diagnosis and timely treatment of EES are pivotal for a favorable prognosis., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Zheng, Zhou, Luo, Zhang, Tu and Min.)