原发性心脏黏液肉瘤并发多发性脑动脉瘤及脑转移1例报告并文献复习。

Chang Gung medical journal Pub Date : 2011-05-01
Tsung-Han Lee, Shun-Chen Huang, Thung-Ming Su, Ka-Yen Yang, Cheng-Shyuan Rau
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摘要

心脏的原发性肿瘤是罕见的。恶性心脏黏液瘤,又称黏液肉瘤,约占原发性心脏恶性肿瘤的6%。恶性心脏黏液瘤发生脑转移极为罕见;只有三例报告,其中两例包括尸检研究。本文所描述的病例是首次报道的脑转移合并起源于原发性心脏黏液肉瘤的多发性脑动脉瘤。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尚未完全确定。目前的假设倾向于来自心脏黏液瘤的肿瘤物质栓塞到外周动脉的血管内,随后在血管壁上增殖。这将导致内膜下组织(如内部弹性层)的削弱,从而导致动脉瘤的形成。心脏黏液肉瘤的预后很差。虽然恶性肿瘤很可能是由间充质细胞发展而来,但它们很难用任何方式(手术、化疗、放疗或移植)治疗,因为这些肿瘤在诊断时通常已经广泛扩散。
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Multiple cerebral aneurysms and brain metastasis from primary cardiac myxosarcoma: a case report and literature review.

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.

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