Vidaillet H J, Seward J B, Fyke F E, Su W P, Tajik A J
Br Heart J. 1987 Mar;57(3):247-55. doi: 10.1136/hrt.57.3.247.
From January 1954 to December 1985 cardiac myxoma was diagnosed in 75 patients at the Mayo Clinic. The clinical presentation was typical in 70 cases and was referred to as "sporadic myxoma". Forty four other cases of cardiac myxomas (five from the Mayo Clinic) presented with a combination of distinctive clinical features and these cases are described as "syndrome myxoma". The patients with syndrome myxoma were younger (mean age, 25 vs 56 years) and had unusual skin freckling (68%), associated benign non-cardiac myxomatous tumours (57%), endocrine neoplasms (30%), and a high frequency of familial cardiac myxoma (25%) and familial endocrine tumours (14%). The two types of cardiac tumour were different (syndrome vs sporadic): atrial location, 87% vs 100%; ventricular location, 13% vs 0%; single tumour, 50% vs 99%; multiple tumours, 50% vs 1%; and recurrent tumour, 18% vs 0%. It is concluded that patients with syndrome myxoma represent a distinctive subgroup in which there are important clinical, surgical, and genetic implications. More importantly, syndrome myxoma appears to be only one expression of a much larger disease entity.
1954年1月至1985年12月期间，梅奥诊所诊断出75例心脏黏液瘤患者。其中70例临床表现典型，被称为“散发性黏液瘤”。另有44例心脏黏液瘤患者（5例来自梅奥诊所）表现出一系列独特的临床特征，这些病例被描述为“综合征性黏液瘤”。综合征性黏液瘤患者更年轻（平均年龄25岁，而散发性黏液瘤患者为56岁），有异常皮肤雀斑（68%）、相关的非心脏良性黏液瘤性肿瘤（57%）、内分泌肿瘤（30%），且家族性心脏黏液瘤（25%）和家族性内分泌肿瘤（14%）的发生率较高。这两种类型的心脏肿瘤存在差异（综合征性与散发性）：位于心房的比例分别为87%和100%；位于心室的比例分别为13%和0%；单发肿瘤的比例分别为50%和99%；多发肿瘤的比例分别为50%和1%；复发性肿瘤的比例分别为18%和0%。结论是，综合征性黏液瘤患者代表了一个独特的亚组，具有重要的临床、手术和遗传学意义。更重要的是，综合征性黏液瘤似乎只是一个更大疾病实体的一种表现形式。
