BACKGROUND Epithelioid angiosarcoma(EA)is an aggressive,malignant endothelial-cell tumor of vascular or lymphatic origin.EA often arises from deep soft tissues such as pleura,breast,bone and gastrointestinal tract.It ...BACKGROUND Epithelioid angiosarcoma(EA)is an aggressive,malignant endothelial-cell tumor of vascular or lymphatic origin.EA often arises from deep soft tissues such as pleura,breast,bone and gastrointestinal tract.It usually affects patients aged 60-70 years and is associated with high recurrence and metastasis rates with surgical resection as the primary treatment of choice.Overall survivals are generally poor,ranging from 6 to 16 months.More than 50%of patients died of disease within 2 to 3 years of diagnosis.CASE SUMMARY We present a rare case of EA of the cervical spine causing a C6 pathological fracture complicated by severe kyphosis.The patient received C4-7 posterior laminectomy and C2/3/4/7/T1 transpedicular screw fixation,followed by anterior C5-6 corpectomy with allograft bone fusion and cervical plate fixation.Postoperative radiotherapy was administered without delay.However,the patient died of rapidly progressive acute respiratory distress syndrome 3 weeks after the second surgery.CONCLUSION EA with spinal involvement is extremely rare.Early detection and cord decompression may prevent neurological deterioration and preserve better quality of life.展开更多
目的探讨Ⅰ型神经纤维瘤病伴重度脊柱侧后凸畸形的临床特征、诊疗策略及预后。方法回顾性分析上海交通大学医学院附属新华医院收治的1例20岁女性Ⅰ型神经纤维瘤病伴脊柱侧后凸畸形患者的临床资料,结合《Ⅰ型神经纤维瘤病临床诊疗专家共...目的探讨Ⅰ型神经纤维瘤病伴重度脊柱侧后凸畸形的临床特征、诊疗策略及预后。方法回顾性分析上海交通大学医学院附属新华医院收治的1例20岁女性Ⅰ型神经纤维瘤病伴脊柱侧后凸畸形患者的临床资料,结合《Ⅰ型神经纤维瘤病临床诊疗专家共识(2021版)》的诊断标准,总结其临床表现、影像学特征及分阶段手术治疗效果。同时,以“neurofibromatosis type 1 AND spinal deformity”及“Ⅰ型神经纤维瘤病和脊柱侧后凸畸形”为关键词,检索PubMed、Web of Science数据库及万方数据库、中国知网,筛选2010—2023年符合纳入排除标准的相关文献,归纳总结诊疗要点及预后特征。结果患者为20岁女性,自幼全身散布咖啡牛奶斑(≥6处),背部有10 cm×10 cm丛状神经纤维瘤,腋窝及腹股沟区多发皮下结节。脊柱侧后凸病程11年,影像学显示Cobb角145°,伴椎体扇形变及肋骨扭曲。双下肢肌力Ⅳ级,踝阵挛阳性。经过头颅-骨盆牵引(halo-gravity traction,HGT),Cobb角为85°,实施后路经椎弓根截骨矫形术(pedicle subtraction osteotomy,PSO)与全椎弓根螺钉固定术,术后脊柱在冠状面和矢状面上保持良好平衡。随访2年,患者症状显著改善。共纳入2010—2023年公开发表的文献5篇进行分析,涉及117例伴脊柱侧后凸畸形的Ⅰ型神经纤维瘤病患者,这些患者均具有丛状神经纤维瘤。脊柱筛查间隔尚未达成共识,手术多采用后路截骨结合椎弓根固定,而丛状神经纤维瘤常采用切除和椎板切除术。前后关节融合术对营养不良性颈椎后凸畸形的矫正效果最佳。结论Ⅰ型神经纤维瘤病伴脊柱侧后凸畸形的治疗需遵循“牵引-截骨矫形-神经功能保护”的综合策略。PSO联合全椎弓根固定能够有效矫正畸形并维持脊柱稳定性,多学科协作与术中神经电生理监测是减少并发症的关键。展开更多
Emerging two-dimensional MXenes have been extensively studied in a wide range of fields thanks to their superior electrical and hydrophilic attributes as well as excellent chemical stability and mechanical flexibility...Emerging two-dimensional MXenes have been extensively studied in a wide range of fields thanks to their superior electrical and hydrophilic attributes as well as excellent chemical stability and mechanical flexibility.Among them,the ultrahigh electrical conductivity(σ)and tunable band structures of benchmark Ti_(3)C_(2)T_(x) MXene demonstrate its good potential as thermoelectric(TE)materials.However,both the large variation ofσreported in the literature and the intrinsically low Seebeck coefficient(S)hinder the practical applications.Herein,this study has for the first time systematically investigated the TE properties of neat Ti_(3)C_(2)T_(x) films,which are finely modulated by exploiting different dispersing solvents,controlling nanosheet sizes and constructing composites.First,deionized water is found to be superior for obtaining closely packed MXene sheets relative to other polar solvents.Second,a simultaneous increase in both S andσis realized via elevating centrifugal speed on MXene aqueous suspensions to obtain small-sized nanosheets,thus yielding an ultrahigh power factor up to~156μW m^(-1) K^(-2).Third,S is significantly enhanced yet accompanied by a reduction inσwhen constructing MXene-based nanocomposites,the latter of which is originated from the damage to the intimate stackings of MXene nanosheets.Together,a correlation between the TE properties of neat Ti_(3)C_(2)T_(x) films and the stacking of nanosheets is elucidated,which would stimulate further exploration of MXene TEs.展开更多
Osteosarcoma(OS)is the most prevalent type of primary malignant bone cancer and currently lacks effective targeted treatments.Increasing evidence indicates that SOX2 overexpression is a primary driver of OS.By screeni...Osteosarcoma(OS)is the most prevalent type of primary malignant bone cancer and currently lacks effective targeted treatments.Increasing evidence indicates that SOX2 overexpression is a primary driver of OS.By screening a small-molecule kinase inhibitor library,we identified AKT as a kinase essential for robust SOX2 expression in OS cells.AKT was found to be frequently overexpressed in OS and positively correlated with SOX2 protein levels.We demonstrated that AKT has no effect on SOX2 transcription but promotes SOX2 protein stability.Mechanistically,AKT binds to and phosphorylates SOX2 at T116,preventing SOX2 ubiquitination and proteasome-dependent degradation by ubiquitin E3 ligases UBR5 and STUB1.Moreover,we found that AKT-SOX2 axis is a significant modulator of cancer stemness and chemoresistance and that the combination of AKT inhibitor MK2206 and cisplatin resulted in a synergistic and potent inhibition of OS tumor growth in the PDX model.In conclusion,we identified a critical role for AKT in promoting SOX2 overexpression,tumor stemness,and chemoresistance in OS,and provided evidence that targeting AKT combined with chemotherapy may hold promise for treating refractory OS.展开更多
文摘BACKGROUND Epithelioid angiosarcoma(EA)is an aggressive,malignant endothelial-cell tumor of vascular or lymphatic origin.EA often arises from deep soft tissues such as pleura,breast,bone and gastrointestinal tract.It usually affects patients aged 60-70 years and is associated with high recurrence and metastasis rates with surgical resection as the primary treatment of choice.Overall survivals are generally poor,ranging from 6 to 16 months.More than 50%of patients died of disease within 2 to 3 years of diagnosis.CASE SUMMARY We present a rare case of EA of the cervical spine causing a C6 pathological fracture complicated by severe kyphosis.The patient received C4-7 posterior laminectomy and C2/3/4/7/T1 transpedicular screw fixation,followed by anterior C5-6 corpectomy with allograft bone fusion and cervical plate fixation.Postoperative radiotherapy was administered without delay.However,the patient died of rapidly progressive acute respiratory distress syndrome 3 weeks after the second surgery.CONCLUSION EA with spinal involvement is extremely rare.Early detection and cord decompression may prevent neurological deterioration and preserve better quality of life.
文摘目的探讨Ⅰ型神经纤维瘤病伴重度脊柱侧后凸畸形的临床特征、诊疗策略及预后。方法回顾性分析上海交通大学医学院附属新华医院收治的1例20岁女性Ⅰ型神经纤维瘤病伴脊柱侧后凸畸形患者的临床资料,结合《Ⅰ型神经纤维瘤病临床诊疗专家共识(2021版)》的诊断标准,总结其临床表现、影像学特征及分阶段手术治疗效果。同时,以“neurofibromatosis type 1 AND spinal deformity”及“Ⅰ型神经纤维瘤病和脊柱侧后凸畸形”为关键词,检索PubMed、Web of Science数据库及万方数据库、中国知网,筛选2010—2023年符合纳入排除标准的相关文献,归纳总结诊疗要点及预后特征。结果患者为20岁女性,自幼全身散布咖啡牛奶斑(≥6处),背部有10 cm×10 cm丛状神经纤维瘤,腋窝及腹股沟区多发皮下结节。脊柱侧后凸病程11年,影像学显示Cobb角145°,伴椎体扇形变及肋骨扭曲。双下肢肌力Ⅳ级,踝阵挛阳性。经过头颅-骨盆牵引(halo-gravity traction,HGT),Cobb角为85°,实施后路经椎弓根截骨矫形术(pedicle subtraction osteotomy,PSO)与全椎弓根螺钉固定术,术后脊柱在冠状面和矢状面上保持良好平衡。随访2年,患者症状显著改善。共纳入2010—2023年公开发表的文献5篇进行分析,涉及117例伴脊柱侧后凸畸形的Ⅰ型神经纤维瘤病患者,这些患者均具有丛状神经纤维瘤。脊柱筛查间隔尚未达成共识,手术多采用后路截骨结合椎弓根固定,而丛状神经纤维瘤常采用切除和椎板切除术。前后关节融合术对营养不良性颈椎后凸畸形的矫正效果最佳。结论Ⅰ型神经纤维瘤病伴脊柱侧后凸畸形的治疗需遵循“牵引-截骨矫形-神经功能保护”的综合策略。PSO联合全椎弓根固定能够有效矫正畸形并维持脊柱稳定性,多学科协作与术中神经电生理监测是减少并发症的关键。
基金supported by the China Postdoctoral Science Foundation(grant No.2024M750511,J.T.)National Key R&D Program of China(grant No.2022YFB3603804,Y.Z.)National Natural Science Foundation of China(NSFC)under grant Nos.82172470(C.X.)and 22375050(Z.L.).
文摘Emerging two-dimensional MXenes have been extensively studied in a wide range of fields thanks to their superior electrical and hydrophilic attributes as well as excellent chemical stability and mechanical flexibility.Among them,the ultrahigh electrical conductivity(σ)and tunable band structures of benchmark Ti_(3)C_(2)T_(x) MXene demonstrate its good potential as thermoelectric(TE)materials.However,both the large variation ofσreported in the literature and the intrinsically low Seebeck coefficient(S)hinder the practical applications.Herein,this study has for the first time systematically investigated the TE properties of neat Ti_(3)C_(2)T_(x) films,which are finely modulated by exploiting different dispersing solvents,controlling nanosheet sizes and constructing composites.First,deionized water is found to be superior for obtaining closely packed MXene sheets relative to other polar solvents.Second,a simultaneous increase in both S andσis realized via elevating centrifugal speed on MXene aqueous suspensions to obtain small-sized nanosheets,thus yielding an ultrahigh power factor up to~156μW m^(-1) K^(-2).Third,S is significantly enhanced yet accompanied by a reduction inσwhen constructing MXene-based nanocomposites,the latter of which is originated from the damage to the intimate stackings of MXene nanosheets.Together,a correlation between the TE properties of neat Ti_(3)C_(2)T_(x) films and the stacking of nanosheets is elucidated,which would stimulate further exploration of MXene TEs.
基金supporting by the National Natural Science Foundation of China(Grant No.82172964,82141105,82072961,82403066)Shanghai Pujiang Talent Program(2020PJD062).
文摘Osteosarcoma(OS)is the most prevalent type of primary malignant bone cancer and currently lacks effective targeted treatments.Increasing evidence indicates that SOX2 overexpression is a primary driver of OS.By screening a small-molecule kinase inhibitor library,we identified AKT as a kinase essential for robust SOX2 expression in OS cells.AKT was found to be frequently overexpressed in OS and positively correlated with SOX2 protein levels.We demonstrated that AKT has no effect on SOX2 transcription but promotes SOX2 protein stability.Mechanistically,AKT binds to and phosphorylates SOX2 at T116,preventing SOX2 ubiquitination and proteasome-dependent degradation by ubiquitin E3 ligases UBR5 and STUB1.Moreover,we found that AKT-SOX2 axis is a significant modulator of cancer stemness and chemoresistance and that the combination of AKT inhibitor MK2206 and cisplatin resulted in a synergistic and potent inhibition of OS tumor growth in the PDX model.In conclusion,we identified a critical role for AKT in promoting SOX2 overexpression,tumor stemness,and chemoresistance in OS,and provided evidence that targeting AKT combined with chemotherapy may hold promise for treating refractory OS.
