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一种具有特征性组织学表现且可能存在遗传性甲状腺球蛋白异常的腺瘤样甲状腺肿变体。

A variant of adenomatous goiter with characteristic histology and possible hereditary thyroglobulin abnormality.

作者信息

Yoshida S, Takamatsu J, Kuma K, Murakami Y, Sakane S, Katayama S, Tarutani O, Ohsawa N

机构信息

First Department of Internal Medicine, Osaka Medical College, Japan.

出版信息

J Clin Endocrinol Metab. 1996 May;81(5):1961-6. doi: 10.1210/jcem.81.5.8626865.

DOI:10.1210/jcem.81.5.8626865
PMID:8626865
Abstract

A variant type of adenomatous goiter was identified in 24 of 2160 patients with adenomatous goiter who underwent thyroidectomy. The characteristics of the thyroid gland in these 24 patients included large goiter, small follicles, scant colloid, and columnar follicular cells containing yellow-green granules on hematoxylin-eosin staining. The thyroid gland was slightly orange-red, and electron microscopic examination showed abundant lysosomes with colloid droplets. When comparing the features of this group with those of 24 patients with common adenomatous goiter, the incidence of familial predisposition to thyroid diseases in the former group was higher. The age at the time of detection of goiter was lower, i.e. 17 +/- 15 vs. 44 +/- 17 yr (P < 0.001, variant type vs. common type), the serum total T4 concentrations were lower (84 +/- 21 vs. 103 +/- 18 nmol/L; P < 0.01), and the serum TSH concentrations were higher (2.4 +/- 2.1 vs. 1.0 +/- 0.9 mU/L; P < 0.01). Thyroid radioiodine uptake was remarkably increased (49 +/- 22 vs. 16 +/- 9%; P < 0.001), and lower levels of serum thyroglobulin were noted (33 +/- 51 vs. 484 +/- 603 micrograms/L; P < 0.01). The thyroglobulin content was low in the thyroid gland studied. The data suggest that the etiology of this variant type of goiter is a hereditary abnormality in thyroglobulin synthesis, and this type of goiter may be distinguished from common adenomatous goiter by the characteristic morphology of the thyroid gland in addition to clinical findings.

摘要

在2160例行甲状腺切除术的腺瘤性甲状腺肿患者中,发现了24例变异型腺瘤性甲状腺肿。这24例患者甲状腺的特征包括甲状腺肿大、滤泡小、胶体少,苏木精-伊红染色可见柱状滤泡细胞含黄绿色颗粒。甲状腺略呈橙红色,电镜检查显示有丰富的含胶体小滴的溶酶体。将该组患者的特征与24例普通腺瘤性甲状腺肿患者的特征进行比较时,前一组甲状腺疾病家族易感性的发生率更高。甲状腺肿发现时的年龄更低,即17±15岁对44±17岁(P<0.001,变异型对普通型),血清总T4浓度更低(84±21对103±18nmol/L;P<0.01),血清促甲状腺激素(TSH)浓度更高(2.4±2.1对1.0±0.9mU/L;P<0.01)。甲状腺放射性碘摄取显著增加(49±22对16±9%;P<0.001),血清甲状腺球蛋白水平更低(33±51对484±603μg/L;P<0.01)。所研究的甲状腺中甲状腺球蛋白含量低。数据表明,这种变异型甲状腺肿的病因是甲状腺球蛋白合成的遗传性异常,除临床发现外,这种类型的甲状腺肿可通过甲状腺的特征形态与普通腺瘤性甲状腺肿相区分。

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引用本文的文献

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A clinically euthyroid child with a large goiter due to a thyroglobulin gene defect: clinical features and genetic studies.一名因甲状腺球蛋白基因缺陷导致甲状腺肿大的临床甲状腺功能正常儿童:临床特征及遗传学研究
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Congenital hypothyroid goiter with deficient thyroglobulin. Identification of an endoplasmic reticulum storage disease with induction of molecular chaperones.伴有甲状腺球蛋白缺乏的先天性甲状腺功能减退性甲状腺肿。一种内质网贮积病的鉴定及分子伴侣的诱导。
J Clin Invest. 1996 Dec 15;98(12):2838-44. doi: 10.1172/JCI119112.