合并尿崩症的成人甲状腺朗格汉斯细胞组织细胞增生症临床特点和诊断经验

Clinical Characteristics and Diagnostic Experience of Adult Thyroid Langerhans Cell Histiocytosis with Diabetes Insipidus

目的分析归纳甲状腺朗格汉斯细胞组织细胞增生症(LCH)的临床特点,分享诊断经验。方法回顾性分析2015年1月至2022年1月复旦大学附属华山医院内分泌科收治的6例病理确诊甲状腺LCH患者的临床资料,分析甲状腺超声和18 F FDG-PET/CT影像学特点。结果6例(2男4女)确诊年龄18~58岁,均因尿崩症就诊于内分泌科,MRI显示垂体柄增粗。中枢性甲减2例,甲功正常4例;甲状腺抗体阳性3例。甲状腺超声提示3类结节3例,4类结节2例,结节性甲状腺肿1例。结节超声特点:均为低回声;5例边界清晰;4例回声分布不均匀;5例形态欠规则;均未见钙化。18 F FDG-PET/CT显示为高摄取结节(SUVmax值>10);手术切除病例确诊4例,粗针穿刺活检病理确诊2例。6例患者确诊时除垂体外,累及肝脏和胸腺各2例,累及肺和骨各1例。系统治疗后4例甲状腺结节缩小,2例伴肝脏累及者病情进展快速未行评估。结论甲状腺LCH超声特点为分布欠均匀的低回声结节,边界清但形态欠规则,不伴钙化;18 F FDG-PET/CT为高摄取。垂体柄增粗患者伴甲状腺低回声欠规则结节时优选甲状腺结节粗针穿刺联合CD1a和langerin染色以助诊断。

Objective To analyze the clinical characteristics of thyroid LCH to enhance understanding of the disease.Methods We retrospectively studied the clinical data from six thyroid LCH patients who hospitalized in Huashan Hospital Affiliated to Fudan University from January 2015 to January 2022.We analyzed the ultrasound and 18 F FDG-PET/CT imaging characteristics of thyroid LCH.Results The six patients diagnosed(2 males and 4 females)were between 18 and 58 years old.All patients had diabetes insipidus.MRI revealed thickened pituitary stalk.Two cases had central hypothyroidism,while four cases euthyroidism.Three cases tested positive for thyroid antibodies.Ultrasound showed thyroid nodules of TI-RADS 3 in three cases,TI-RADS 4 in two cases,and 1 with nodular goiter.Ultrasound showed that all sic cases indicated low echogenicity,5 of which clear boundaries,4 of which uneven echo distribution,5 of which irregular shape,and noen has calcification.18 F FDG-PET/CT indicated high uptake nodules with SUVmax values all above 10.4 cases were diagnosed by surgical excision and the other 2 by coarse-needle aspiration biopsy.When diagnosed,two cases had liver and thymus involvement respectively.One case had lung and bone involvement respectively.After treatment,4 cases showed that nodular goiter shrank,while the other two with liver involvement progressed fast and no assessment made.Conclusions Thyroid LCH presented low echogenicity,clear boundaries,irregular shape,without calcification,and high uptake in 18 F FDG-PET/CT.A definite diagnosis of pituitary stalk thicking accompanied by thyroid nodules,especially those with hypoechoic and irregular nodules,can be achieved by coarse-needle aspiration biopsy and langerin-specific pathological staining.