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[多发性复发性心脏黏液瘤。它是一种家族性疾病吗?]

[Multiple and recurrent cardiac myxomas. Is it a familial disease?].

作者信息

Duveau D, Baron O, Jegou B, Remadi J P, Michaud J L, Despins P, De Lajartre A Y, Gaillard F

机构信息

Clinique chirurgicale thoracique cardiaque et vasculaire, Hôpital G.-et-R.-Laënnec, C.H.U. Nantes.

出版信息

Chirurgie. 1993;119(6-7):357-61.

PMID:7805495
Abstract

From 1964 to 1992, 56 patients with cardiac myxoma have been operated on. Sixty seven operations have been made during which 85 myxomas have been excised. Five patients had been reoperated from recurrent myxoma: 4 patients 2 times, 1 patient one time. In this population we observe 7 cases of multiple myxomas implanted 7 times in many cavities. The study of this series displays a lot of risk factors: implantation outside the left atrium, multiple myxomas in one or several cavities, true recurrence of myxoma, young age of the patient, associated lesions as part of the Carney's complex. In this series, 13 patients, representing 5 families, have one or more of these risk factors. in two of them, a familial character has been demonstrated. Analysis of these special kinds of cardiac myxomas displays very closed analogy between familial myxomas and high risk group. These findings suggest that recurrent and/or multiple and/or familial myxoma represent the same entity.

摘要

1964年至1992年期间,56例心脏黏液瘤患者接受了手术治疗。共进行了67次手术,切除了85个黏液瘤。5例患者因黏液瘤复发接受了再次手术:4例患者接受了2次手术,1例患者接受了1次手术。在这组病例中,我们观察到7例多发性黏液瘤,分别在多个腔室中植入了7次。对该系列病例的研究显示出许多危险因素:瘤体植入左心房以外、一个或多个腔室中的多发性黏液瘤、黏液瘤的真正复发、患者年龄较小、作为卡尼综合征一部分的相关病变。在这个系列中,13例患者(代表5个家族)具有一种或多种这些危险因素。其中两例已证实具有家族性特征。对这些特殊类型的心脏黏液瘤的分析显示,家族性黏液瘤与高危组之间存在非常密切的相似性。这些发现表明,复发性和/或多发性和/或家族性黏液瘤代表同一实体。

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