Objective: To explore the clinical and radiological features of myelin oligodendrocyte glycoprotein (MOG) antibody associated disease. Methods: The clinical data of 22 MOG antibody associated disease cases treated in the Department of Neurology, Qilu Hospital of Shandong University from January 2017 to June 2019 were retrospectively analyzed. The clinical data of MOG antibody associated disease were summarized, including clinical and imaging features. Results: Of the 22 included patients with MOG antibody associated disease, the average age was 38.5 years, 13 were male and nine were female. Among them, 11 cases manifested as aquaporin-4 (AQP4)-negative neuromyelitis optica spectrum disorder (NMOSD), four cases optic neuritis, two cases transverse myelitis, one case acute disseminated encephalomyelitis (ADEM), two cases cortical encephalitis and two cases vestibular neuronitis. Magnetic resonance imaging (MRI) results showed that multiple anatomical areas were involved. Among the nine patients with optic nerve involvement, five patients had longitudinally extensive optic nerve lesions, which were longitudinally enhanced. In eight patients, MRI lesions in the spinal cord showed mostly long or short segments involvement, involving 2-5 spinal cord segments. Five cases involved the cervical spinal cord, six cases involved the thoracic spinal cord, and one case involved the lumbar spinal cord. Brain MRI abnormalities were found in 13 cases and the lesions were mostly patchy and point-shaped. MRI lesions demonstrated T₂ hyperintensity and some of them could be strengthened, which may involve the basal ganglia, thalamus, radiographic crown, frontal temporal lobe, brain stem and other parts. Among them, 16 patients were sensitive to high-dose intravenous/oral methylprednisolone in the acute phase. Seven patients had recurrence after two months to two years of follow-up. Conclusions MOG antibody associated disease include multiple manifestations. Among them, AQP4-negative NMOSD is the most common form. The clinical manifestations of patients showed diversity. Imaging is characterized by multiple parts involvement such as optic nerve, spinal cord, and brain. Most patients are sensitive to high-dose intravenous/oral methylprednisolone, and have a good prognosis in the acute phase, but some patients may relapse.

Objective:Limb salvage treatment for osteosarcoma in the distal tibiais challenging and the incidence of postoperative complications is high. To prove that the use of autologous bone inactivation to replant the ankle joint has a satisfactory clinical effect.Methods:This study retrospectively studied 6 cases (4 males and 2 females) of the osteosarcoma of the distal tibia from June 2018 to April 2019 in our center. The average case age was 16.8 years. All cases were first diagnosed in the center, and classic osteosarcoma was confirmed by biopsy. Before the operation, he received 4 courses of neoadjuvant chemotherapy. The tumor was resected under general anesthesia, and the tumor bone was inactivated and replanted for internal fixation and reconstruction. During the operation, the tumor segment was completely removed, and after a series of treatments, it was immersed in hypertonic saline at 65 °C for 45 min. After removal, the bone defect was filled with bone cement, and internal fixation materials such as plate screws and anchors were placed. Finally, soft tissue was rebuilt after the inactivated bone internal fixation complex (autograft-fixation composite, AFC) was replanted. Patients were treated with plaster for 3 months after surgery. Chemotherapy continued 2 weeks after surgery. Follow-up X-ray of the lower tibia were taken at 3 months, 6 months, and 12 months after the operation to evaluate the MSTS score and ankle function score (AOFAS) to evaluate the ankle function.Results:The patients were followed up for an average of 20 months. At 12 months after operation, no complications such as deep infection, mechanical failure of internal fixation, or local tumor recurrence occurred. The MSTS score averaged 26.7±2.6 points. The AOFAS average was 82.3±8.5 points. The VAS average is 2.7 points. Boneunionwasobserved in 5 cases during follow-up. All cases were satisfied with the treatment results.Conclusion:Limb salvage treatment of distal tibia osteosarcoma is more difficult. This study proves that the use of tumor bone inactivation and internal fixation to reconstruct the ankle joint can not only cure the tumor, but also has satisfactory limb function. It is an ideal strategy for limb salvage reconstruction.

To report the short-term clinical outcome of three cases of distal tibial osteosarcoma treated with a novel 3D-printed ankle fusion prosthesis for limb preservation. The patients were admitted to the Department of Bone Tumor, Shanghai General Hospital from January 2020 to June 2021, with one male and two female cases, aged 18, 12, and 14 years, respectively, all diagnosed with distal tibial osteosarcoma (Ennecking stage IIb). A new self-designed ankle fusion prosthesis was used to perform osteosarcoma resection and prosthetic reconstruction of the distal tibia. The operation time, blood loss, postoperative American Orthopedic Foot and Ankle Society Score (AOFAS) and ankle range of motion were recorded. All the 3 patients successfully completed the operation and were followed up for 22 months, 18 months and 12 months, respectively. The operation time was 140 min, 110 min and 200 min, and the blood loss was 200 ml, 200 ml and 350 ml, respectively. At the last follow-up, the AOFAS were 86, 90 and 95 points, and the range of motion of ankle flexion and extension were 30°, 15° and 30°. There was no local recurrence or lung metastasis at the last follow-up. The novel 3D-printed ankle fusion prosthesis in the distal tibia is safe and effective for the reconstruction of bone defects after resection of osteosarcoma in the distal tibia, and the early postoperative function is satisfactory.

Objective:To investigate the mid-to-long term therapeutic effects of allogeneic bone composite prosthesis reconstruction in patients with large bone defects after the resection of distal femoral tumors.Methods:From June 2013 to December 2018, a total of 19 patients with malignant tumors of the distal femur who underwent reconstruction with allogeneic bone composite prosthesis in the Department of Bone Tumor, Shanghai General Hospital were retrospectively collected. There were 10 males and 9 females, aged 22.3±11 years (range, 11-42 years). The mean body mass index was 19.3±3.4 kg/m 2 (range, 14-27 kg/m 2). There were 18 cases of osteosarcoma and 1 case of Ewing's sarcoma. According to Ennecking staging, there were 17 cases of stage IIB and 2 cases of stage III. The intraoperative blood loss and operation time were recorded, and the prosthesis and patient survival conditions and postoperative complications were observed. The limb function was evaluated by the Musculoskeletal Tumor Society (MSTS) 93 function score. Results:All patients successfully completed the operation. The operation time was 187.3±39.8 min (range, 110-260 min), the intraoperative blood loss was 284.9±87.0 ml (range, 200-500 ml), and the blood transfusion volume was 327±213 ml (range, 100-800 ml). The remaining length of the proximal femur was 153.7±26.6 mm (range, 93-190 mm), and the length of allogeneic bone was 84.1±24.6 mm (range, 39-134 mm). Among the 19 patients, 9 patients (47%) achieved bony union with an average healing time of 16.7±4.8 months (range, 10-25 months), and 7 patients had delayed healing with an average healing time of 18.4±4.0 months (range, 15-25 months). The remaining 10 cases were nonunion between allogeneic bone and host bone. All patients were followed up for 80.7±20.2 months (range, 56-121 months). During the follow-up, 3 cases died due to pulmonary metastasis of bone tumors, and the time of death was 57 months, 63 months, and 59 months after surgery, respectively. At the last follow-up, the patient survival rate was 84% (16/19), and the MSTS 93 function score of the 16 patients was (24.3±2.4) points (range, 21-28 points), with an excellent rate of 100% (16/16). Seven patients underwent revision surgery, 3 cases were aseptic loosening, 3 cases were prosthesis stem fracture at the junction of the allograft bone and the host bone, and 1 case was periprosthetic infection, among which the patient with periprosthetic infection had poor local soft tissue conditions due to preoperative radiotherapy, and the infection was controlled after two revision surgeries. Five cases were revised with allogeneic bone composite prosthesis, and 2 cases were revised with short-stem giant prosthesis with cortical steel plate or locking nail. After revision, the remaining length of the proximal femur was 143.4±31 mm (range, 91-175 mm), and the length of the allograft bone was 92.6±26.6 mm (range, 61-123 mm). The 7 revised patients were still in follow-up. There were no cases of pulmonary infection, nerve injury, deep vein thrombosis or other complications after surgery.Conclusion:The survival period of patients after the surgery to reconstruct large bone defects following the resection of malignant tumors at the distal end of the femur using allogeneic bone composite prosthesis is satisfactory, and the limb function is good. However, the incidence of prosthesis complications is high, which can be reconstructed through revision.

Objective:To explore the construction and application methods of multicenter bone tumor-specific database.Methods:Experts from multiple centers including Shanghai General Hospital, Shanghai Changzheng Hospital, Zhongshan Hospital, Shanghai Sixth People's Hospital, Ruijin Hospital, Fudan University Shanghai Cancer Center and Shanghai Ninth People's Hospital established a standard dataset for bone tumors through research and discussion. Clinical data will be automatically collected and standardized according to standard fields. A database will be built and a users' interface will be developed to ensure secure data storage, while providing services such as exporting raw data, visualizing statistical analysis, establishing clinical queue research projects, et al. Finally, the bone tumor database will be shared by integrating with the Shenkang's Big Data Platform to achieve multi-center data integration.Results:A standard data set for bone tumors containing 603 fields has been established and published. An automated data collection system for bone tumors has been established, including complete data collection, data collation and visualization functions. The data categories include modules such as patients' electronic case information, laboratory information on blood routine, biochemistry and tumor markers, imaging information, surgery information, pathology information and radiotherapy records. Personal information such as patients' names and ID numbers are desensitized and encrypted and can be exported for further research. From 2015 to 2023, the total number of bone tumor cases collected in the database was 10,789. From 2015 to 2019, 112 cases of the osteosarcoma cohort were retrospectively analyzed for admission, with a statistical 5-year survival rate of 68%.Conclusion:A regional bone tumor specialty big data network and data sharing platform has been established, along with data sharing mechanisms and standards including data standards, security standards, and quality evaluation standards. This provides data and efficient new solutions for the construction of China's bone tumor database, as well as a research and development platform for standardized diagnosis and treatment of bone tumors and new technologies.