甲状腺朗格汉斯细胞组织细胞增生症一例

A Case of Langerhans Cell Histiocytosis of the Thyroid Gland

摘要: 本文报道一例罕见的甲状腺朗格汉斯细胞组织细胞增生症；患者，女，72岁，2月前因声音嘶哑就诊于我院，行甲状腺彩超示：甲状腺右侧体积明显增大并回声不均致气管受压。故行手术切除，术中探查见甲状腺右侧叶弥漫肿大，大小约15 × 10 × 8 cm，完整切除肿物送病理检查。镜下见上皮样细胞或组织细胞样细胞组成，胞质丰富，细胞核大而不规则，轻度不典型，可见核沟，具有空泡状或“咖啡豆”外观，核仁不明显，间质嗜酸性粒细胞浸润。局灶区域可见瘤细胞增大，核仁明显，异型性显著，核分裂象增多。免疫组化染色：肿瘤细胞同时表达CD1a、S100蛋白和langerin (CD207)。病理诊断：“甲状腺”朗格汉斯细胞组织细胞增生症，局灶肉瘤样改变。在罕见部位发生的此类疾病容易误诊、漏诊，需要提高对此类疾病的认识，形态结合免疫组化做出准确诊断。

Abstract: This article reports a rare case of Langerhans cell histiocytosis of the thyroid gland; the patient, a 72-year-old female, presented to our hospital 2 months ago due to hoarseness, and thyroid gland ultrasound showed that the right side of the thyroid gland was significantly enlarged and had uneven echogenicity, resulting in tracheal compression. Surgical resection was therefore performed. Intraoperative exploration showed that the right lobe of the thyroid gland was diffusely enlarged with a size of about 15 × 10 × 8 cm, and the complete resection of the mass was sent for pathological examination. Under the microscope, epithelioid or histiocyte-like cells with abundant cytoplasm, large and irregular nuclei, mild atypia, visible nuclear grooves, vacuolated or “coffee bean” appearance, inconspicuous nucleoli, and interstitial eosinophilic infiltration were observed. The tumor cells are enlarged in focal areas, with prominent nucleoli, marked heterogeneity, and increased nuclear schizophrenia. Immunohistochemical staining: tumor cells expressed both CD1a, S100 protein and langerin (CD207). Pathologic diagnosis: Langerhans cell histiocytosis of the “thyroid gland” with focal sarcomatoid changes. The occurrence of this disease in a rare site can be easily misdiagnosed and underdiagnosed, and there is a need to raise awareness of this disease and make an accurate diagnosis by combining morphology with immunohistochemistry.