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
A 66-year-old female with a 1-month history of increasing fatigue, dyspnea on exertion, and palpitations presented with clinical signs of heart failure. Chest computed tomography (CT) revealed a large, mobile left atr...A 66-year-old female with a 1-month history of increasing fatigue, dyspnea on exertion, and palpitations presented with clinical signs of heart failure. Chest computed tomography (CT) revealed a large, mobile left atrial (LA) mass attached to the mitral valve causing severe mitral stenosis and mitral regurgitation. The mass was surgically debulked and the mitral valve was replaced. Pathology revealed a poorly differentiated malignant spindle cell neoplasm with diffuse nuclear positivity of MDM2 and multifocal positivity of CDK4, consistent with intimal sarcoma. This case seeks to describe an uncommon presentation of a rare malignancy, and the surgical and medical management of the disease.展开更多
Malignant cardiac tumors are rare and challenging entity. Experience in treating these aggressive and lethal tumors is minimal and management protocols are not well defined and have to rely on case reports, and person...Malignant cardiac tumors are rare and challenging entity. Experience in treating these aggressive and lethal tumors is minimal and management protocols are not well defined and have to rely on case reports, and personal experiences. Most effective therapy for primary cardiac malignancy is a complete surgical resection and the role of chemoradiotherapy is still evolving.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.
文摘A 66-year-old female with a 1-month history of increasing fatigue, dyspnea on exertion, and palpitations presented with clinical signs of heart failure. Chest computed tomography (CT) revealed a large, mobile left atrial (LA) mass attached to the mitral valve causing severe mitral stenosis and mitral regurgitation. The mass was surgically debulked and the mitral valve was replaced. Pathology revealed a poorly differentiated malignant spindle cell neoplasm with diffuse nuclear positivity of MDM2 and multifocal positivity of CDK4, consistent with intimal sarcoma. This case seeks to describe an uncommon presentation of a rare malignancy, and the surgical and medical management of the disease.
文摘Malignant cardiac tumors are rare and challenging entity. Experience in treating these aggressive and lethal tumors is minimal and management protocols are not well defined and have to rely on case reports, and personal experiences. Most effective therapy for primary cardiac malignancy is a complete surgical resection and the role of chemoradiotherapy is still evolving.
