MYXOID FIBROADENOMA AND ALLIED CONDITIONS (MYXOMATOSIS) OF THE BREAST - A HERITABLE DISORDER WITH SPECIAL ASSOCIATIONS INCLUDING CARDIAC AND CUTANEOUS MYXOMAS
MYXOID FIBROADENOMA AND ALLIED CONDITIONS (MYXOMATOSIS) OF THE BREAST - A HERITABLE DISORDER WITH SPECIAL ASSOCIATIONS INCLUDING CARDIAC AND CUTANEOUS MYXOMAS
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DOI：
10.1097/00000478-199108000-00001
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发表时间：
1991-08-01
影响因子：
5.6
通讯作者：
TOORKEY, BC
中科院分区：
文献类型：
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作者：
CARNEY, JA;TOORKEY, BC
在145例粘液瘤、斑点状色素沉着、内分泌过度活跃和沙瘤性黑色素性神经鞘瘤的复合体患者中，31例（21%）有乳腺病变。这26名女性和5名男性的年龄范围为6至64岁（平均30岁）。五名患者出现乳房症状。在21例（81%）女性中，病理检查发现良性间叶病变。其特征为小叶内大量基质的积聚，使间质变为非常疏松的粘液样组织。该变化涉及单个小叶（小叶粘液样变化）、小小叶群（结节性粘液样变化）和大小叶聚集体（粘液样纤维腺瘤）;小叶间基质受影响程度较低。8例患者（38%）的病变为多中心和双侧。由于乳腺粘液样病变是家族性的，在复合体中是常见的，并且在组织学上与复合体中的心脏和皮肤粘液瘤相似，因此它们无疑是复合体的组成部分和病理标志物。它们是6例患者（19%）的综合征的表现特征。因此，在病理检查中发现粘液样乳腺病变应引起对该综合征的怀疑，并对受累患者（及其主要亲属）进行相应的评估。
Among 145 patients with the complex of myxomas, spotty pigmentation, endocrine overactivity, and psammomatous melanotic schwannomas, 31 (21%) had mammary lesions. The ages of these 26 females and five males ranged from 6 to 64 years (mean, 30 years). Five patients had breast symptoms. In 21 (81%) of the females, benign mesenchymal lesion(s) were detected pathologically. These were characterized by accumulations of large amounts of ground substance in the lobules that alterated the stroma to a very loose and myxoid tissue. The change involved single lobules (lobular myxoid change), small groups of lobules (nodular myxoid change), and large aggregates of lobules (myxoid fibroadenoma); the interlobular stroma was affected to a lesser degree. The lesions were multicentric and bilateral in eight patients (38%). Because the myxoid breast lesions were familial, were frequent findings in the complex, and were similar histologically to the cardiac and cutaneous myxomas in the complex, they undoubtedly are a component and a pathologic marker of the complex. They were the presenting feature of the complex in six patients (19%). Therefore, discovery of the myxoid breast lesions on pathologic examination should raise suspicion of the complex, and affected patients (and their primary relatives) should be evaluated accordingly.