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罕见的甲状腺肿病因:甲状腺朗格汉斯细胞组织细胞增生症。

A rare cause of goiter: Langerhans cell histiocytosis of the thyroid.

机构信息

Department of Endocrinology, the First Affiliated Hospital of Chongqing Medical University, Chongqing, China.

出版信息

Endocr J. 2012;59(1):47-54. doi: 10.1507/endocrj.ej11-0243. Epub 2011 Oct 21.

Abstract

Goiter is a very common clinical problem; however, Langerhans cell histiocytosis (LCH) with thyroid involvement that presents as a goiter is very rare. In this article, we report one case of thyroid LCH. An 18-year-old male patient presented with goiter, polyuria, polydipsia, and lymphadenectasis of the neck, and LCH was confirmed by a lymph node biopsy and pathological investigation. Without a thyroidectomy, the goiter shrank after nine cycles of chemotherapy. In addition, we summarize the reported thyroid LCH cases in the literature from the last 10 years. LCH usually involves other organs, such as the lungs, bones, skin, pituitary gland, and lymph nodes. Thyroid LCH is more common in adults than in children, and it may coexist with a thyroid carcinoma. Without any unique thyroid manifestations, either clinically or by imaging, it is difficult to distinguish thyroid LCH from other thyroid diseases. Pathology is the gold standard for the diagnosis of LCH. A fine needle aspiration biopsy (FNAB) may help to diagnosis LCH, although sometimes it leads to misdiagnosis. Chemotherapy is recommended for multi-system LCH. Younger patients with widespread disease or who are non-responsive to chemotherapy have poor outcomes.

摘要

甲状腺肿是一种非常常见的临床问题;然而,伴有甲状腺受累的朗格汉斯细胞组织细胞增生症(LCH)表现为甲状腺肿则非常罕见。本文报道了 1 例甲状腺 LCH。1 例 18 岁男性患者因甲状腺肿、多尿、多饮和颈部淋巴结肿大就诊,通过淋巴结活检和病理检查证实为 LCH。未经甲状腺切除术,9 个周期化疗后甲状腺肿缩小。此外,我们总结了过去 10 年文献中报道的甲状腺 LCH 病例。LCH 通常累及其他器官,如肺、骨骼、皮肤、垂体和淋巴结。与儿童相比,成人中甲状腺 LCH 更为常见,且可能与甲状腺癌并存。无论是临床还是影像学表现,均无任何独特的甲状腺表现,因此很难将甲状腺 LCH 与其他甲状腺疾病区分开来。病理学是 LCH 诊断的金标准。细针抽吸活检(FNAB)有助于诊断 LCH,但有时会导致误诊。多系统 LCH 推荐化疗。广泛性疾病或对化疗无反应的年轻患者预后不良。

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