Clinical Characteristics and Diagnostic Experience of Adult Thyroid Langerhans Cell Histiocytosis with Diabetes Insipidus
10.12376/j.issn.2097-0501.2023.03.005
- VernacularTitle: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
- Author:
Qian WANG
1
;
Quanya SUN
1
;
Min HE
1
;
Shuo ZHANG
1
;
Boni XIANG
1
;
Qiufan LI
2
;
Yong WANG
2
;
Xialing ZHANG
3
;
Tianling DING
4
;
Hongying YE
1
Author Information
1. Department of Endocrinology, Huashan Hospital Affiliated to Fudan University, Shanghai 200040, China.
2. Department of Ultrasound, Huashan Hospital Affiliated to Fudan University, Shanghai 200040, China.
3. Department of Pathology, Huashan Hospital Affiliated to Fudan University, Shanghai 200040, China.
4. Department of Hematology, Huashan Hospital Affiliated to Fudan University, Shanghai 200040, China.
- Publication Type:Journal Article
- Keywords:
Langerhans cell histiocytosis;
thyroid;
pituitary stalk thickness
- From:
JOURNAL OF RARE DISEASES
2023;2(3):346-352
- CountryChina
- Language:Chinese
-
Abstract:
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 18F 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.18F 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 18F 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.
