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
BACKGROUND Primary intimal sarcoma of the pulmonary artery is a rare malignant tumor originating from the pulmonary artery,which has a low incidence rate and is easily misdiagnosed as pulmonary embolism.There is no st...BACKGROUND Primary intimal sarcoma of the pulmonary artery is a rare malignant tumor originating from the pulmonary artery,which has a low incidence rate and is easily misdiagnosed as pulmonary embolism.There is no standard protocol for the treatment of primary intimal sarcoma of the pulmonary artery.CASE SUMMARY This study reports a patient with primary intimal sarcoma of the pulmonary artery who was admitted to our hospital in 2017.The clinical characteristics,diagnosis,treatment and outcome of the patient were retrospectively analyzed.The patient was a Chinese Han male aged 44 years.He had three consecutive episodes of syncope,and was thus admitted to a local hospital.Computed tomography pulmonary angiography showed multiple lesions with abnormal densities in the pulmonary trunk,left pulmonary artery,mediastinum and pericardium,which were consistent with recurrence after tumor resection.He underwent surgery,and was pathologically diagnosed with intimal sarcoma of the pulmonary artery.He relapsed 3 mo after surgery,and apatinib was administered.His condition was stable after 4 mo,with tolerable and controllable adverse reactions.He subsequently died 19 mo after surgery.CONCLUSION Primary intimal sarcoma of the pulmonary artery has no specific clinical or imaging manifestations.The diagnosis of this disease depends on histopathology and immunohistochemistry,and has a poor clinical prognosis.Surgical treatment is currently a favorable option for primary intimal sarcoma of the pulmonary artery,and targeted therapy may provide new insights for the development of effective treatment methods.展开更多
The long arm of human chromosome 12 contains a region that has been found to be amplified in a number of different tumors, including osteosarcomas and soft tissue sarcomas. There are more than 5 genes located in this ...The long arm of human chromosome 12 contains a region that has been found to be amplified in a number of different tumors, including osteosarcomas and soft tissue sarcomas. There are more than 5 genes located in this area such as CDK2, CDK4, WNT1, MDM2 and WNTIOb. CDK4 gene consists of eight exons, of which the start codon is located in the beginning of exon 2 and the stop codon in the a member of the Ser-Thr catalytic domain extends beginning of exon 8. CDK4 is protein kinase family and its from amino acid 6 to 295.展开更多
Pulmonary artery intimal sarcoma （PALS） is a very rare but lethal disease, firstly described by Mandelstarnmin 1923.1 Since then, less than 300 cases have been reported worldwide. Due to similar clinical presentatio...Pulmonary artery intimal sarcoma （PALS） is a very rare but lethal disease, firstly described by Mandelstarnmin 1923.1 Since then, less than 300 cases have been reported worldwide. Due to similar clinical presentations, it is very difficult to distinguish with pulmonary thromboembolism （PTE）, leading to inappropriate treatments such as anticoagulation and thrombolysis.2-5Although with improvement of imaging modalities, the diagnosis of PAIS is still based on pathological examination, and the majority of specimens are taken by surgery or autopsy.展开更多
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BACKGROUND Primary intimal sarcoma of the pulmonary artery is a rare malignant tumor originating from the pulmonary artery,which has a low incidence rate and is easily misdiagnosed as pulmonary embolism.There is no standard protocol for the treatment of primary intimal sarcoma of the pulmonary artery.CASE SUMMARY This study reports a patient with primary intimal sarcoma of the pulmonary artery who was admitted to our hospital in 2017.The clinical characteristics,diagnosis,treatment and outcome of the patient were retrospectively analyzed.The patient was a Chinese Han male aged 44 years.He had three consecutive episodes of syncope,and was thus admitted to a local hospital.Computed tomography pulmonary angiography showed multiple lesions with abnormal densities in the pulmonary trunk,left pulmonary artery,mediastinum and pericardium,which were consistent with recurrence after tumor resection.He underwent surgery,and was pathologically diagnosed with intimal sarcoma of the pulmonary artery.He relapsed 3 mo after surgery,and apatinib was administered.His condition was stable after 4 mo,with tolerable and controllable adverse reactions.He subsequently died 19 mo after surgery.CONCLUSION Primary intimal sarcoma of the pulmonary artery has no specific clinical or imaging manifestations.The diagnosis of this disease depends on histopathology and immunohistochemistry,and has a poor clinical prognosis.Surgical treatment is currently a favorable option for primary intimal sarcoma of the pulmonary artery,and targeted therapy may provide new insights for the development of effective treatment methods.
文摘The long arm of human chromosome 12 contains a region that has been found to be amplified in a number of different tumors, including osteosarcomas and soft tissue sarcomas. There are more than 5 genes located in this area such as CDK2, CDK4, WNT1, MDM2 and WNTIOb. CDK4 gene consists of eight exons, of which the start codon is located in the beginning of exon 2 and the stop codon in the a member of the Ser-Thr catalytic domain extends beginning of exon 8. CDK4 is protein kinase family and its from amino acid 6 to 295.
文摘Pulmonary artery intimal sarcoma （PALS） is a very rare but lethal disease, firstly described by Mandelstarnmin 1923.1 Since then, less than 300 cases have been reported worldwide. Due to similar clinical presentations, it is very difficult to distinguish with pulmonary thromboembolism （PTE）, leading to inappropriate treatments such as anticoagulation and thrombolysis.2-5Although with improvement of imaging modalities, the diagnosis of PAIS is still based on pathological examination, and the majority of specimens are taken by surgery or autopsy.
