Back to Search Start Over

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

Authors :
WANG Qian
SUN Quanya
HE Min
ZHANG Shuo
XIANG Boni
LI Qiufan
WANG Yong
ZHANG Xialing
DING Tianling
YE Hongying
Source :
罕见病研究, Vol 2, Iss 3, Pp 346-352 (2023)
Publication Year :
2023
Publisher :
Editorial Office of Journal of Rare Diseases, 2023.

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.

Details

Language :
Chinese
ISSN :
20970501
Volume :
2
Issue :
3
Database :
Directory of Open Access Journals
Journal :
罕见病研究
Publication Type :
Academic Journal
Accession number :
edsdoj.30a4b634b4ba491aaae36e9c94295dcc
Document Type :
article
Full Text :
https://doi.org/10.12376/j.issn.2097-0501.2023.03.005