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目的探讨甲状腺原发性朗格汉斯组织细胞增生症的临床病理学特征及鉴别诊断要点。方法对1例甲状腺原发的朗格汉斯组织细胞增生症进行组织形态学及免疫组化分析,并复习相关文献。结果患者女性,55岁。5年前无明显诱因出现心慌、多汗等症状,双侧甲状腺进行性、弥漫性肿大。术中见病变境界欠清晰,与周围组织广泛粘连,触之易出血。镜下见肿瘤细胞弥漫浸润甲状腺组织并累及周围横纹肌,可见核沟,背景可见嗜酸性粒细胞。免疫组化示肿瘤细胞Langerin、CD1α和S-100强(+)。术后随访18个月,无复发。结论甲状腺朗格汉斯组织细胞增生症非常罕见,确诊需要组织病理学及免疫组化的证据,临床易与低分化癌混淆。
Objective To investigate the clinicopathological characteristics and differential diagnosis of primary Langerhans cell histiocytosis in thyroid. Methods One case of Langerhans cell histiocytosis with primary thyroid was analyzed by histomorphology and immunohistochemistry, and the related literatures were reviewed. Results Patients Female, 55 years old. 5 years ago no obvious incentive to palpitation, sweating and other symptoms, bilateral thyroid progressive diffuse enlargement. Intraoperative see the realm of the disease is not clear, with the surrounding tissue extensive adhesion, easy to touch the bleeding. Microscope see tumor cells diffuse infiltration of thyroid tissue and surrounding striated muscle involvement, showing a nuclear ditch, eosinophils can be seen in the background. Immunohistochemistry showed that Langerin, CD1α and S-100 were strong (+). The patients were followed up for 18 months without recurrence. Conclusions The Langerhans cell histiocytosis in the thyroid gland is very rare. The diagnosis requires histopathological and immunohistochemical evidence, which is easily confused with poorly differentiated cancers.