Thyroid pathologic findings in patients with Cowden disease.
Thyroid pathologic findings in patients with Cowden disease.
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DOI：
10.1016/s1092-9134(99)80011-2
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发表时间：
1999-12-01
影响因子：
2
通讯作者：
Longy, M
中科院分区：
文献类型：
--
作者：
Harach, H R;Soubeyran, I;Longy, M
我们描述了六名女性和五名男性考登病患者甲状腺的组织学发现。患者年龄9至43岁（平均年龄26岁）。该综合征中突出的甲状腺病变是多中心滤泡性腺瘤和腺瘤性（实质、增生性）结节，显示出广泛的非特异性细胞结构模式。多个微小细胞灶，即所谓的微腺瘤，也是一个特征。还出现由嗜酸细胞或透明细胞组成的特定病变、具有透明样小梁腺瘤特征的肿瘤和腺脂肪瘤。两例病例除了多个良性滤泡细胞增殖外，还显示出滤泡癌。滤泡性癌发生的年龄较大，且尺寸大于临床上显着的良性结节性病变，表明肿瘤进展。所有肿瘤均显示甲状腺球蛋白免疫反应性，且降钙素呈阴性。多发性腺瘤性甲状腺肿或多发性滤泡性腺瘤的组织学发现，特别是在儿童和年轻人中，应提醒病理学家和医生注意遗传性状的可能性，例如考登病，及其对家庭筛查的影响。肿瘤通常是良性且界限清楚，但由于多中心性和复发或进展为癌的风险增加，应提倡甲状腺全切除术。
We describe the histologic findings in thyroid glands from six female and five male patients with Cowden disease. The patients were aged 9 to 43 years (mean age, 26 years). The salient thyroid lesions in this syndrome were multicentric follicular adenomas and adenomatous (parenchymatous, hyperplastic) nodules showing a wide range of nonspecific cytoarchitectural patterns. Multiple tiny cellular foci, so-called microadenomas, were also a feature. Specific lesions composed of oxyphil or clear cells, a tumor with features of hyalinizing trabecular adenoma, and an adenolipoma also occurred. Two cases showed a follicular carcinoma in addition to multiple benign follicular cell proliferations. The follicular carcinomas occurred at an older age and were larger in size than the clinically significant benign nodular lesions, suggesting tumor progression. All tumors showed thyroglobulin immunoreactivity and were negative for calcitonin. The histologic findings of a multiple adenomatous goiter or multiple follicular adenomas, particularly in children and young adults, should alert the pathologist and physician to the possibility of an inherited trait, such as Cowden disease, with its implications for family screening. The tumors are usually benign and well demarcated, but, because of multicentricity and increased risk of recurrence or progression to carcinoma, total thyroidectomy should be advocated.