Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival.

Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival.
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DOI:
10.1016/j.humpath.2008.01.019
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发表时间:
2008-09
期刊:
影响因子:
3.3
通讯作者:
Narula, Navneet
Narula, Navneet
中科院分区:
医学3区
文献类型:
--
作者:
Zhang, Paul J.;Brooks, John S.;Goldblum, John R.;Do, Brian Yoder;Seethata, Raja;Pawel, Bruce;Gorman, Joseph H.;Gorman, Robert C.;Huang, Jui-Han;Acker, Michael;Narula, Navneet

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尽管与软组织对应的心脏肉瘤相比,心脏肉瘤很少见,但它们是第二常见的心脏原发肿瘤。在报告的数百例病例中,大多数是基于尸检系列。本文回顾了一系列27例心脏肉瘤的临床病理特征,并与17例患者的术后随访资料进行了比较。其中血管肉瘤6例,粘液纤维肉瘤6例,恶性周围神经鞘膜瘤3例,平滑肌肉瘤3例,滑膜肉瘤2例,上皮样血管内皮瘤1例,软骨肉瘤1例,骨肉瘤1例,低分化肉瘤4例。年龄和体型范围很广,略有女性偏好。发生于心房/肺血管20例,室壁4例,二尖瓣1例,心外膜/心包2例。有一种轻微的左倾倾向。组织学分级低4例,中3例,高20例。6例高级别和1例低级别肿瘤还接受了辅助化疗和/或放射治疗。17例有随访资料的患者中,12例高级别肿瘤患者中有6例死亡(4例在初次手术后5天内死亡,1例在21个月内死亡,1例在131个月内死亡),1例中级别肿瘤患者和4例低级别肿瘤患者在随访结束时均存活,无疾病迹象。在心脏肉瘤中,肿瘤分级似乎是预后的重要因素。在最初的手术中幸存下来的患者获得了长期生存。
Although cardiac sarcomas are rare in comparison to their soft tissue counterparts, they are the second most common type of primary cardiac neoplasm. Of the few hundred cases reported, most has been based on autopsy series. A series of 27 cardiac sarcomas removed at surgery for curative and diagnostic intent were reviewed for clinicopathologic features with correlation to available postoperative follow-up data in 17 patients. There were 6 angiosarcomas, 6 myxofibrosarcomas, 3 malignant peripheral nerve sheath tumors, 3 leiomyosarcomas, 2 synovial sarcomas, 1 epithelioid hemangioendothelioma, 1 chondrosarcoma, 1 osteosarcoma, and 4 poorly differentiated sarcomas. There was a wide age and size range with slight female predilection. There were 20 cases that arose in the atria/pulmonary vessels, 4 in the ventricles, 1 in mitral valve, and 2 in epi/pericardium. There was a slight left predilection. The histologic grade was low in 4, moderate in 3, and high in 20 cases. Six high-grade and 1 low-grade tumors were also treated with adjuvant chemotherapy and/or radiation. In 17 patients with follow-up data, 6 of 12 patients with high-grade tumor died (4 within 5 days of the initial surgery, 1 in 21 months, and 1 in 131 months), and 1 patient with moderate-grade tumor and all 4 patients with low-grade tumor were alive without evidence of disease at the end of follow-up. Tumor grade appeared to be prognostically important in cardiac sarcoma. Long survival was achieved in patients who survived the initial surgery well.
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影响因子: --
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