Department of Endocrinology, Key Laboratory of Endocrinology of National Health Commission, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Front Endocrinol (Lausanne). 2022 Sep 30;13:1013616. doi: 10.3389/fendo.2022.1013616. eCollection 2022.
Langerhans cell histiocytosis (LCH) is a rare disease caused by the clonal expansion of CD1a+/CD207+ LCH cells. The thyroid involvement in LCH has mostly been described in case reports.
We retrospectively evaluated the clinical characteristics, diagnosis, and treatment of 27 children and adult patients with thyroid LCH in our center between 2010 and 2021.
The incidence of thyroid LCH was 14.00% (7/50) in children and 10.10% (20/198) in adults, respectively. Among patients with thyroid involvement, 81.5% presented with diabetes insipidus (DI) as the first symptom, and 51.9% complained of neck swelling or mass. Children and adults with thyroid LCH had higher frequencies of the hypothalamic-pituitary axis (HPA) (children: 100% vs. 62.8%, P=0.05; adult: 95% vs. 42.1%, P<0.001), the lung (children: 85.7% vs. 25.6%, P=0.004; adult: 70% vs. 50.6%, P=0.099), and a lower frequency of bone (children: 14.3% vs. 55.8%, P=0.049; adult: 45% vs. 73.6%, P=0.008) involvement than patients without thyroid involvement. Patients with thyroid LCH had a higher frequency of primary hypothyroidism and a lower frequency of euthyroidism than patients without it. The two major types of ultrasound imaging were diffuse (55%) and nodular type (45%). The standardized uptake value of thyroid on 18-F-fluorodeoxyglucose positron emission tomography/computed tomography was 5.3-12.8. The diagnoses were confirmed using thyroid aspiration (54.5%) or surgery (45.5%). In addition, thyroid LCH combined with papillary thyroid carcinoma was not rare (2/27).
Thyroid involvement in LCH is not rare. Furthermore, identifying thyroid involvement can facilitate the pathological diagnosis of LCH. Therefore, the possibility of thyroid LCH should be fully investigated in patients with DI, primary hypothyroidism, abnormal thyroid ultrasound results, and multi-system disease. In addition, thyroid aspiration can confirm suspected thyroid LCH. Finally, special attention should be paid to evaluating HPA and pulmonary involvement in thyroid LCH.
朗格汉斯细胞组织细胞增生症（LCH）是一种由 CD1a+/CD207+LCH 细胞克隆性扩张引起的罕见疾病。甲状腺受累在 LCH 中的描述主要见于病例报告。
我们回顾性评估了 2010 年至 2021 年间我们中心 27 例儿童和成人甲状腺 LCH 患者的临床特征、诊断和治疗。
甲状腺 LCH 的发生率分别为儿童的 14.00%（7/50）和成人的 10.10%（20/198）。在甲状腺受累的患者中，81.5%以尿崩症（DI）为首发症状，51.9%诉颈部肿胀或肿块。儿童和成人甲状腺 LCH 的下丘脑-垂体轴（HPA）受累频率更高（儿童：100%比 62.8%，P=0.05；成人：95%比 42.1%，P<0.001），肺部受累频率更高（儿童：85.7%比 25.6%，P=0.004；成人：70%比 50.6%，P=0.099），而骨骼受累频率更低（儿童：14.3%比 55.8%，P=0.049；成人：45%比 73.6%，P=0.008）。与无甲状腺受累的患者相比，甲状腺 LCH 患者原发性甲状腺功能减退症的发生率更高，甲状腺功能正常的发生率更低。两种主要的超声成像类型为弥漫型（55%）和结节型（45%）。18-氟-脱氧葡萄糖正电子发射断层扫描/计算机断层扫描甲状腺的标准摄取值为 5.3-12.8。甲状腺抽吸（54.5%）或手术（45.5%）确诊。此外，甲状腺 LCH 合并甲状腺乳头状癌并不少见（2/27）。
甲状腺受累在 LCH 中并不少见。此外，确定甲状腺受累有助于 LCH 的病理诊断。因此，在 DI、原发性甲状腺功能减退症、异常甲状腺超声结果和多系统疾病患者中，应充分考虑甲状腺 LCH 的可能性。此外，甲状腺抽吸可证实疑似甲状腺 LCH。最后，应特别注意评估甲状腺 LCH 的 HPA 和肺部受累。
