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原发性心脏黏液肉瘤伴多发脑动脉瘤及脑转移:一例报告并文献复习

Multiple cerebral aneurysms and brain metastasis from primary cardiac myxosarcoma: a case report and literature review.

作者信息

Lee Tsung-Han, Huang Shun-Chen, Su Thung-Ming, Yang Ka-Yen, Rau Cheng-Shyuan

机构信息

Division of Trauma, Department of Surgery, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan.

出版信息

Chang Gung Med J. 2011 May-Jun;34(3):315-9.

Abstract

Primary neoplasms of the heart are rare. Malignant cardiac myxoma, or so-called myxosarcoma, accounts for about 6% of primary malignant cardiac tumors. Cerebral metastasis of malignant cardiac myxoma is extremely rare; only three cases have been reported and two of them included an autopsy study. The case described herein is the first reported brain metastasis combined with multiple cerebral aneurysms originating from primary cardiac myxosarcoma. The true incidence of cerebral myxomatous aneurysm is unknown and the pathogenesis of myxomatous aneurysm formation has not been fully defined. The current hypothesis favors that tumor materials from cardiac myxomas embolize into the vasa vasorum of the peripheral arteries and subsequently prolifere in the vessel wall. This then leads to a weakening of subintimal tissue, such as the internal elastic lamina, with subsequent aneurysm formation. The prognosis of cardiac myxosarcoma is very poor. Although malignancies most likely develop from the mesenchymal cells, they are difficult to treat with any modality (operation, chemotherapy, radiotherapy or transplantation) because these tumors have usually undergone extensive spread by the time the diagnosis is made.

摘要

原发性心脏肿瘤很罕见。恶性心脏黏液瘤,即所谓的黏液肉瘤,约占原发性恶性心脏肿瘤的6%。恶性心脏黏液瘤的脑转移极为罕见;仅报道过3例,其中2例包含尸检研究。本文所述病例是首例报道的源自原发性心脏黏液肉瘤的脑转移合并多发性脑动脉瘤。脑黏液瘤性动脉瘤的真实发病率尚不清楚,黏液瘤性动脉瘤形成的发病机制也尚未完全明确。目前的假说认为,心脏黏液瘤的肿瘤物质栓塞到外周动脉的滋养血管,随后在血管壁中增殖。这进而导致内膜下组织(如内弹性膜)变薄弱,随后形成动脉瘤。心脏黏液肉瘤的预后非常差。尽管恶性肿瘤很可能起源于间充质细胞,但由于这些肿瘤在确诊时通常已发生广泛扩散,所以难以用任何方式(手术、化疗、放疗或移植)进行治疗。

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