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
Targeted precision neuroendocrine neoplasms (NEN) surgery may represent an innovative, yet promising, development of surgical procedures to achieve a radical resection of the primary tumour (by intra-operative confirm...Targeted precision neuroendocrine neoplasms (NEN) surgery may represent an innovative, yet promising, development of surgical procedures to achieve a radical resection of the primary tumour (by intra-operative confirmation of tumour-free margins) and to improve the identification of small sized lesions (that might otherwise be missed by either pre-operative functional imaging, ultrasound or manual palpation during surgery). From a practical point of view, radioguided surgery (RGS) requires a close collaboration between the nuclear medicine and the surgical teams, so that a small amount of radiopharmaceutical is intravenously injected in the patient right before surgery. The tumour lesions’ emitted radiation is then detected by a hand-held probe by the surgeon intra-operatively.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Targeted precision neuroendocrine neoplasms (NEN) surgery may represent an innovative, yet promising, development of surgical procedures to achieve a radical resection of the primary tumour (by intra-operative confirmation of tumour-free margins) and to improve the identification of small sized lesions (that might otherwise be missed by either pre-operative functional imaging, ultrasound or manual palpation during surgery). From a practical point of view, radioguided surgery (RGS) requires a close collaboration between the nuclear medicine and the surgical teams, so that a small amount of radiopharmaceutical is intravenously injected in the patient right before surgery. The tumour lesions’ emitted radiation is then detected by a hand-held probe by the surgeon intra-operatively.
