Cardiac Sarcomas An Update

被引:90
作者
Orlandi, Augusto [1 ]
Ferlosio, Amedeo [1 ]
Roselli, Mario [2 ]
Chiariello, Luigi [3 ]
Spagnoli, Luigi G. [1 ]
机构
[1] Tor Vergata Univ, Dept Anat Pathol, I-00133 Rome, Italy
[2] Tor Vergata Univ, Dept Cardiac Surg, I-00133 Rome, Italy
[3] Tor Vergata Univ, Dept Oncol, I-00133 Rome, Italy
关键词
Tumors of the heart; Classification; Diagnosis; Therapy; Pleomorphic sarcoma; Angiosarcoma; SOFT-TISSUE SARCOMAS; PHASE-II TRIAL; ADJUVANT CHEMOTHERAPY; HEART-TRANSPLANTATION; SURGICAL PATHOLOGY; LEFT ATRIUM; TUMORS; ANGIOSARCOMA; EXPERIENCE; THERAPY;
D O I
10.1097/JTO.0b013e3181e59a91
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
Primary cardiac sarcomas are rare and represent 20% of all primary cardiac tumors Symptoms depend on the chambers and the cardiac structures involved Transthoracic echocardiography is commonly used to identify a cardiac mass The diagnosis of cardiac sarcoma requires adequate sampling and the careful use of ancillary diagnostic techniques In the most recent histologic classification, angiosarcoma is the most common malignant tumor of the heart with recognizable differentiation Undifferentiated sarcomas account for one-third of all cardiac sarcomas and have been incorporated in the malignant fibrous histiocytoma/pleomorphic sarcoma subgroup Elective cardiac sarcoma therapy includes complete surgical excision when possible. followed by radio and chemotherapeutic regimen, the latter preferably containing anthracyclines, ifosfamide. or taxanes Prognosis of cardiac sarcomas is very poor, with mean survival ranging from 9 6 to 16 5 months A less-aggressive course seems related to the left atrium location, a low histologic grading with scarce cellular pleomorphism and low-mitotic activity, absence of necrosis, myxoid tumor appearance. and absence of metastasis at diagnosis
引用
收藏
页码:1483 / 1489
页数:7
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