[Multiple myxomata: about one case].

C Jayle, S Franco, Le Bris, G Lefort, P Corbi
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Abstract

Among the primitive cardiac tumours, myxoma is the most common. This benign tumour is sometimes described in the context of Carney's syndrome, in which cardiac myxoma, cutaneous myxoma, lentigo and pigmentary nevus cutaneous lesions, endocrine disorders, and testicular, thyroid and hypophyseal tumours are associated. The cardiac myxomata observed are multiple, recurrent, and involve the four cardiac chambers, with a peak incidence at 25 years of age. These observations may exist in a familial context, linked to an autosomal dominant genetic factor, localized on the 17q2 chromosome with polymorphism of the PRKAR1a gene. As in the case of sporadic myxoma, rapid surgical treatment with cardio-pulmonary bypass is indicated, bearing in mind the increased risk of thromboembolic phenomena and sudden death from valvular encroachment. We report a case of bi-atrial myxoma observed in the context of Carney's syndrome.

多发黏液瘤:约1例。
在原始心脏肿瘤中,黏液瘤是最常见的。这种良性肿瘤有时在卡尼综合征的背景下被描述,其中心脏黏液瘤、皮肤黏液瘤、小痣和色素痣皮肤病变、内分泌紊乱、睾丸、甲状腺和垂体肿瘤相关。心脏黏液瘤为多发、复发性,累及四个心室,25岁为发病高峰。这些观察结果可能存在于家族背景下,与常染色体显性遗传因素有关,该遗传因素位于PRKAR1a基因多态性的17q2染色体上。在散发性黏液瘤的病例中,考虑到血栓栓塞现象和因瓣膜侵犯而猝死的风险增加,建议快速手术治疗并进行心肺搭桥。我们报告一例双心房黏液瘤在卡尼综合征的背景下观察。
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