BACKGROUND Cardiac tumors are rare and complex entities.Surgery represents the cornerstone of therapy,while the role of adjuvant treatment remains unclear and,in case of relapse or metastatic disease,the prognosis is ...BACKGROUND Cardiac tumors are rare and complex entities.Surgery represents the cornerstone of therapy,while the role of adjuvant treatment remains unclear and,in case of relapse or metastatic disease,the prognosis is very poor.Lack of prospective,randomized clinical trials hinders the generation of high level evidence for the optimal diagnostic workup and multimodal treatment of cardiac sarcomas.Herein,we describe the multidisciplinary clinical management and molecular characterization of a rare case of cardiac myxofibrosarcoma in an elderly woman.CASE SUMMARY A 73-year-old woman presented signs and symptoms of acute left-sided heart failure.Imaging examination revealed a large,left atrial mass.With suspicion of a myxoma,she underwent surgery,and symptoms were promptly relieved.Histology showed a cardiac myxofibrosarcoma,a rare histotype of cardiac sarcoma.Eight months later,disease unfortunately relapsed,and after a multidisciplinary discussion,a chemotherapy with doxorubicin and then gemcitabine was started,achieving partial radiologic and complete metabolic response,which was maintained up to 2 years and is still present.This report is focused on the entire clinical path of our patient from diagnosis to follow-up,through surgery and strategies adopted at relapse.Moreover,due to their rarity,very little is known about the molecular landscape of myxofibrosarcomas.Thus,we also performed and described preliminary genome analysis of the tumor tissue to get further insight on mechanisms involved in tumor growth,and to possibly unveil new clinically actionable targets.CONCLUSION We report a case of cardiac myxofibrosarcoma that achieved a very good prognosis due to an integrated surgical,cardiac and oncologic treatment strategy.展开更多
Temporary mechanical circulatory support(tMCS)plays a pivotal role in managing severe or refractory cardiogenic shock(CS),furnishing essential hemodynamic support,and sustaining organ perfusion from hours to weeks,or ...Temporary mechanical circulatory support(tMCS)plays a pivotal role in managing severe or refractory cardiogenic shock(CS),furnishing essential hemodynamic support,and sustaining organ perfusion from hours to weeks,or to even months.In the past decade,the utilization of tMCS has witnessed a signicant increase in American and European countries.Meanwhile in Asia,the newly developed concepts and devices constituting tMCS have also made rapid progress.Nevertheless,the absence of a consensus or standardized guidelines,especially for the Asian population considering inherited polymorphism,is evident.To address this knowledge gap,eight associations/societies and experts in thiseld came up with the present consensus after repeated discussions,more specically for the Asian population.It comprehensively elucidates the commonly employed tMCS devices,criteria for patient selection,indications and contraindications,clinical management,and the optimal timing for implantation and weaning.The goal of this statement is to promote the standardized application of tMCS and make reasonable decisions in clinical practice.Full-length version of the consensus can be seen in Supplementary File,http://links.lww.com/CM9/B883.展开更多
文摘BACKGROUND Cardiac tumors are rare and complex entities.Surgery represents the cornerstone of therapy,while the role of adjuvant treatment remains unclear and,in case of relapse or metastatic disease,the prognosis is very poor.Lack of prospective,randomized clinical trials hinders the generation of high level evidence for the optimal diagnostic workup and multimodal treatment of cardiac sarcomas.Herein,we describe the multidisciplinary clinical management and molecular characterization of a rare case of cardiac myxofibrosarcoma in an elderly woman.CASE SUMMARY A 73-year-old woman presented signs and symptoms of acute left-sided heart failure.Imaging examination revealed a large,left atrial mass.With suspicion of a myxoma,she underwent surgery,and symptoms were promptly relieved.Histology showed a cardiac myxofibrosarcoma,a rare histotype of cardiac sarcoma.Eight months later,disease unfortunately relapsed,and after a multidisciplinary discussion,a chemotherapy with doxorubicin and then gemcitabine was started,achieving partial radiologic and complete metabolic response,which was maintained up to 2 years and is still present.This report is focused on the entire clinical path of our patient from diagnosis to follow-up,through surgery and strategies adopted at relapse.Moreover,due to their rarity,very little is known about the molecular landscape of myxofibrosarcomas.Thus,we also performed and described preliminary genome analysis of the tumor tissue to get further insight on mechanisms involved in tumor growth,and to possibly unveil new clinically actionable targets.CONCLUSION We report a case of cardiac myxofibrosarcoma that achieved a very good prognosis due to an integrated surgical,cardiac and oncologic treatment strategy.
基金National Natural Science Foundation of China(Nos.81974034,81970396,81670351,and 82001701)National Key Research and Development Program(No.2021YFA 1101900)
文摘Temporary mechanical circulatory support(tMCS)plays a pivotal role in managing severe or refractory cardiogenic shock(CS),furnishing essential hemodynamic support,and sustaining organ perfusion from hours to weeks,or to even months.In the past decade,the utilization of tMCS has witnessed a signicant increase in American and European countries.Meanwhile in Asia,the newly developed concepts and devices constituting tMCS have also made rapid progress.Nevertheless,the absence of a consensus or standardized guidelines,especially for the Asian population considering inherited polymorphism,is evident.To address this knowledge gap,eight associations/societies and experts in thiseld came up with the present consensus after repeated discussions,more specically for the Asian population.It comprehensively elucidates the commonly employed tMCS devices,criteria for patient selection,indications and contraindications,clinical management,and the optimal timing for implantation and weaning.The goal of this statement is to promote the standardized application of tMCS and make reasonable decisions in clinical practice.Full-length version of the consensus can be seen in Supplementary File,http://links.lww.com/CM9/B883.