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Frontiers of Medicine

ISSN 2095-0217

ISSN 2095-0225(Online)

CN 11-5983/R

Postal Subscription Code 80-967

2018 Impact Factor: 1.847

Front Med    2013, Vol. 7 Issue (1) : 143-146     DOI: 10.1007/s11684-013-0238-6
CASE REPORT |
A case of thymic Langerhans cell histiocytosis with diabetes insipidus as the first presentation
Xiaoyan Chen1(), Xiaochun Huang1, Yuan Qiu2, Hanzhang Chen2, Yingyu Fu1, Xinchun Li3
1. Department of Endocrinology; 2. Department of Thoracic Surgery; 3. Department of Radiology, First Affiliated Hospital of Guangzhou Medical College, Guangzhou 510120, China
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Abstract  

Langerhans cell histiocytosis (LCH) is an idiopathic group of reactive proliferative diseases linked to aberrant immunity, pathologically characterized by clonal proliferation of Langerhans cells. LCH rarely involves the thymus. We report a case of thymic LCH with diabetes insipidus as the first presentation, without evidence of myasthenia gravis and without evidenced involvement of the skin, liver, spleen, bones, lungs and superficial lymph nodes. This present case may have important clinical implications. In screening for LCH lesions, attention should be attached to rarely involved sites in addition to commonly involved organs. Follow-up and imageological examination are very important to a final diagnosis.

Keywords diabetes insipidus      thymus      Langerhans cell histiocytosis     
Corresponding Authors: Chen Xiaoyan,Email:gzscxy@126.com   
Issue Date: 05 March 2013
URL:  
http://academic.hep.com.cn/fmd/EN/10.1007/s11684-013-0238-6     OR     http://academic.hep.com.cn/fmd/EN/Y2013/V7/I1/143
Fig.1  Contrast-enhanced-T1-weighted MR image of the pituitary (coronal view). Pituitary stalk showed slight thickening and obvious enhancement (arrow). (Image created on September 7, 2010.)
Fig.1  Contrast-enhanced-T1-weighted MR image of the pituitary (coronal view). Pituitary stalk showed slight thickening and obvious enhancement (arrow). (Image created on September 7, 2010.)
Fig.2  Contrast-enhanced-T1-weighted MR image of the pituitary (sagittal view) showed the normal pituitary gland with no abnormal change noticed; pituitary stalk showed slight thickening and obvious enhancement (arrow). (Image created on September 7, 2010.)
Fig.2  Contrast-enhanced-T1-weighted MR image of the pituitary (sagittal view) showed the normal pituitary gland with no abnormal change noticed; pituitary stalk showed slight thickening and obvious enhancement (arrow). (Image created on September 7, 2010.)
Fig.3  T1-weighted MR image indicating the loss of high signal intensity of pituitary posterior lobe (arrow). (Image created on September 7, 2010.)
Fig.3  T1-weighted MR image indicating the loss of high signal intensity of pituitary posterior lobe (arrow). (Image created on September 7, 2010.)
Fig.4  CT scan image of the chest. A small nodule localized at anterior mediastinum (arrow), possibly associated with thymic hyperplasia or thymoma. (Image created on March 29, 2011.)
Fig.4  CT scan image of the chest. A small nodule localized at anterior mediastinum (arrow), possibly associated with thymic hyperplasia or thymoma. (Image created on March 29, 2011.)
Fig.5  MR image of the pituitary: loss of high signal intensity of pituitary posterior lobe (arrow). (Image created on March 29, 2011.)
Fig.5  MR image of the pituitary: loss of high signal intensity of pituitary posterior lobe (arrow). (Image created on March 29, 2011.)
Fig.6  MR image of the pituitary: nodular thickening of the pituitary stalk after six months’ follow-up (arrow). (Image created on March 29, 2011.)
Fig.6  MR image of the pituitary: nodular thickening of the pituitary stalk after six months’ follow-up (arrow). (Image created on March 29, 2011.)
Fig.7  MR image of the pituitary: nodular thickening of the pituitary stalk (arrow), considered to be a benign lesion that was consistent with diabetes insipidus. (Image created on March 29, 2011.)
Fig.7  MR image of the pituitary: nodular thickening of the pituitary stalk (arrow), considered to be a benign lesion that was consistent with diabetes insipidus. (Image created on March 29, 2011.)
Fig.8  A distinct type of cell with focal and light staining cytoplasm, irregular and grooved nuclei (arrow), and 0 to 6 mitotic figures per 10 high-power fields (HPF), was juxtaposed against a backdrop of numerous eosinophils. (Photographed on April 26, 2011.)
Fig.8  A distinct type of cell with focal and light staining cytoplasm, irregular and grooved nuclei (arrow), and 0 to 6 mitotic figures per 10 high-power fields (HPF), was juxtaposed against a backdrop of numerous eosinophils. (Photographed on April 26, 2011.)
Fig.9  Immunohistochemical assay: S-100 (+). (Photographed on April 26, 2011.)
Fig.9  Immunohistochemical assay: S-100 (+). (Photographed on April 26, 2011.)
Fig.10  Immunohistochemical assay: CD1a weakly (+). (Photographed on April 26, 2011.)
Fig.10  Immunohistochemical assay: CD1a weakly (+). (Photographed on April 26, 2011.)
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