原发性肺动脉肉瘤一例

患者女,59岁.因"反复发热1个月余,发现心脏杂音半个月"于2008年11月入院.患者体温最高达39℃,一般为38℃左右.发热前有畏寒,同时身上有红斑,四肢关节疼痛.于外院行抗感染治疗后,效果不明显,体温有反复.体检:于主动脉瓣第一听诊区闻及3/6级收缩期杂音,于肺动脉瓣听诊区闻及3/6级收缩期杂音.     

An unusual primary vascular tumor: intimal sarcoma of the pulmonary artery

Primary sarcomas of great vessels are rare and involve the aorta, pulmonary artery and inferior vena cava. The pathologic classification of these tumors can be made on the location of the sarcoma in relation to the vessel wall, luminal or mural. Luminal sarcomas are usually intimal sarcoma and mural sarcoma are most frequently leiomyosarcoma. The myofibroblastic or endothelial differentiation of these tumors is still debated. We report a case of intimal sarcoma of the pulmonary artery.     

肺动脉内膜肉瘤1例并文献复习

目的探讨血管内膜肉瘤的临床及病理特点。方法对1例肺动脉内膜肉瘤进行光镜、电镜观察和免疫组化检测并复习文献。结果肿瘤位于肺动脉血管腔内,阻塞管腔,并延伸至周围小血管。光镜下肿瘤细胞主要为梭形细胞,散在分布上皮样细胞和多核巨细胞,核分裂象约46／50HPF。电镜观察见细胞内含有丰富的粗面内质网、线粒体,无向特殊细胞分化的特征。免疫组化染色vimentin呈弥漫阳性、α—SMA灶性阳性、HMB-45散在弱阳性,CKpan、CD34、CD31和S-100蛋白等均阴性。结论内膜肉瘤是一种罕见的发生于大血管壁的恶性肿瘤,预后差,免疫表型无特异性,了解该肿瘤发病部位、临床及病理特点有助于做出正确诊断。     

Right pulmonary artery sarcoma

A 57-year-old man presented with cough, chest pain and dyspnea. Peripheral lung opacities detected radiologically on admission were surgically resected and histologically confirmed as pulmonary infarcts. Subsequent radiological examinations revealed a stricture of the right pulmonary artery with evidence of pulmonary hypertension. The patient was commenced on anticoagulant therapy, which resulted in some clinical improvement. A hilar mass detected later was resected together with the right lung. Pathological examination of the hilar mass revealed a tumour within the lumen of the pulmonary artery causing almost total occlusion of the artery. Histology showed angiosarcomatous and osteosarcomatous areas. The patient is well and disease-free 9 months following resection.     

Intimal sarcoma of the pulmonary artery: report of an autopsy case

Primary pulmonary artery sarcomas (PASs) are rare and lethal tumors. They are easily misdiagnosed as chronic pulmonary embolism, mediastinal mass or tumor emboli, which delay a proper treatment. Although the advanced technologies are now increasingly being used, their diagnosis is usually hard to establish preoperatively at the present time. We report here a case of a 68-year-old female with PAS with lung metastases, who firstly presented with symptoms of common cold and anemia. Although a PAS had been suspected, the final diagnosis of pulmonary intimal sarcoma was made only postoperatively by histological and immunohistochemical examination. The patient died 8 months after the operation because of tumor growth progression, despite adjuvant chemotherapy and radiation therapy. Although pulmonary intimal sarcomas are usually of poorly differentiated mesenchymal malignancy, most reported cases are immunohistochemically positive for vimentin, alpha-smooth muscle actin (SMA), and/or desmin, therefore resembling leiomyosarcomas. However, the diagnosis of leiomyosarcoma should not be made on the basis of immunostains in the absence of typical morphologic features, and PAS, like the present case, should be more appropriately classified as intimal sarcoma according to the new WHO Classification of Tumours of Soft Tissue and Bone published in 2002.     

A case of primary pericardial undifferentiated sarcoma

Primary pericardial sarcomas are extremely rare. The authors report a case of a 46-yr-old woman in whom a large mediastinal mass was discovered. The patient presented with cough, dyspnea, and orthopnea. Diagnostic investigations, such as echocardiography, computed tomography, and exploratory thoracotomy provided the evidence of a large mass in the mediastinum, attached by a broad base to the superior portion of the pericardium. A excisional biopsy was performed, and histologic examination of a biopsy specimen showed undifferentiated sarcoma. However, the complete removal of the mass was impossible due to adhesion to the adjacent great vessels. After the completion of the chemotherapy the patient was completely asymptomatic. However, follow-up transesophageal echocardiography showed a residual 3 x 4 cm-sized mass. The patient received the radiotherapy with a total dose of 55 Gy over 6 weeks. At present, there is no evidence of disease progression.     

A case of pulmonary artery intimal sarcoma diagnosed with multislice CT scan with 3D reconstruction

Pulmonary artery intimal sarcoma is a rare highly lethal disease, with additional retrograde extension to pulmonic valve and right ventricle being an extremely rare condition. It is frequently mistaken for pulmonary thromboembolism. We report a case of 64-year-old woman with progressive dyspnea initially suspected and treated for pulmonary thromboembolism. Her helical chest CT scan with 3 dimensional (3D) reconstruction combined with echocardiography revealed a compacting main pulmonary artery mass extending to the right ventricular outflow tract and the right pulmonary artery. After excision of the mass, the patient's condition improved dramatically, and the pathologic findings revealed pulmonary intimal sarcoma. This report emphasizes that helical chest CT with 3D reconstruction can be an important tool to differentiate the characteristics of pulmonary artery lesions, such as intimal sarcoma and thromboembolism.     

肺动脉内膜肉瘤临床病理学观察

目的观察肺动脉内膜肉瘤的临床病理特征,了解其基因改变情况。方法分析中日友好医院2017年1月至2020年1月间行肺动脉内膜剥除术病例60例,收集其中5例肺动脉内膜肉瘤患者临床资料,采用HE染色、免疫组织化学染色及荧光原位杂交进行病理形态学观察,通过RNA测序方法分析肿瘤融合基因改变。结果肺动脉内膜肉瘤检出率为8.3%(5/60),中位年龄49岁,女性多于男性,临床表现不特异。肿瘤由异型梭形细胞或上皮样细胞组成,可出现骨肉瘤及平滑肌肉瘤分化。肿瘤细胞可表达血小板源性生长因子受体α、细胞周期蛋白依赖性激酶4(CDK4)及MDM2,并伴有MDM2、CDK4及表皮生长因子受体基因共扩增。RNA测序检出多种框内融合基因改变。结论肺动脉内膜肉瘤是一类罕见、高度异质性并伴有多基因复杂改变的低~未分化肉瘤,治疗效果不佳,预后差。     

A unique case of pulmonary artery leiomyosarcoma

Primary pulmonary artery leiomyosarcomas are rare, and the diagnosis is usually confused with other, more common, diseases, especially pulmonary embolism. A 52-year-old male, previously healthy, sustained a cardiac arrest. Chest CT-angiography diagnosed a "saddle embolus". Local thrombolysis was tried without any obvious success. At this point, the possibility of neoplasm was entertained. A cardiac MRI showed a nonhomogeneous mass in the proximal pulmonary artery. Successful surgery was performed, and histological examination of the resected mass was consistent with leiomyosarcoma. A follow-up cardiac MRI showed no residual mass. The dilemma associated with diagnosing pulmonary artery leiomyosarcomas will be discussed.     

Primary pulmonary sarcoma with morphologic features of biphasic synovial sarcoma: a case report.

We report an unusual primary case of pulmonary sarcoma that developed in the lung of a 36-year-old woman. The tumor had histologic, immunologic and ultrastructural features identical to those of biphasic synovial sarcoma of the soft tissue. It consisted of an intimate admixture of cytokeratin and epithelial membrane antigen(EMA)-positive neoplastic epithelial cells and vimentin-positive fibroblast-like spindle cells with areas of hyalinization. The patient had a lobectomy and showed no evidence of recurrence or tumor at other sites 15 months after surgery. This case is an useful addition to the small number of published reports on pulmonary synovial sarcoma. The distinctive features of this neoplasm allow it to be different from other types of primary and metastatic malignancies in the lung.     

Myeloid sarcoma of the brain. A case report

We report a case of myeloid sarcoma of the brain mimicking a meningioma on CT scan. The lesion was first morphologically misdiagnosed as a lymphoma, but correctly identified by using immunochemistry with anti-myeloperoxidase, anti-CD68, anti-CD15 antibodies. An acute myeloid leukemia was diagnosed 5 months later. Myeloid sarcoma is frequently mistaken for malignant lymphoma, especially when it presents without leukemic manifestation, even at immunohistochemistry, since both express some leukocyte antigens. Careful evaluation of morphology for evidence of myeloid differentiation, and immunohistochemistry using anti-myeloperoxidase, anti-lysozyme, CD15, CD68 antibodies, should be used to confirm the diagnosis and to rule out lymphoma since the treatment is different.     

Primary pulmonary synovial sarcoma. Apropos of 1 case with cytogenetic study

Synovial sarcoma most commonly occurs in the peri-articular regions of the extremities. We report a case of primary pulmonary monophasic synovial sarcoma. This tumor is extremely rare and shows a particular immunohistochemical pattern of great help for the diagnosis. Cytogenetic study confirm the diagnosis by showing the specific t (X; 18) chromosomal translocation, characteristic of synovial sarcoma in all anatomic locations.