Journal List > Endocrinol Metab > v.26(1) > 1085921

Hong, Ohn, Kim, Hwang-Bo, Kim, Kwon, Lee, Choi, Lee, Cho, Shin, Park, Jang, Cho, Lee, Shin, Yang, and Kim: Clinical Characteristics of Langerhans Cell Histiocytosis with Hypothalamo-Pituitary Involvement

Abstract

Background

Langerhans cell histiocytosis (LCH) is a rare disease that involves a clonal proliferation of Langerhans cells. LCH has a predilection for hypothalamo-pituitary axis (HPA) dysfunction, and this leads to diabetes insipidus (DI) and/or anterior pituitary dysfunction. Here, we describe the endocrine dysfunction and clinical characteristics of adult patients with LCH and we analyzed the differences between an adult-onset type and a childhood-onset type.

Methods

The data was obtained from a retrospective chart review of the patients with LCH that involved the HPA and who attended Seoul National University Hospital. The patients were classified into the adult-onset type (age at the time of diagnosis ≥ 16) and the childhood-onset type (age at the time of diagnosis ≤ 15).

Results

Ten patients (9 males and 1 female) were diagnosed with LCH involving the HPA. Five patients were classified as an adultonset type and the other five patients were classified as a childhood-onset type. The median follow-up duration was 6 (3-12) years for the adult-onset type and 16 (15-22) years for the childhood-onset type. All the patients presented with DI as the initial manifestation of HPA involvement. Four adult-onset patients and three childhood-onset patients had a multi-system disease. Panhypopituitarism developed in three adult-onset patients and in one childhood-onset patient. The pituitary lesion of the three adult-onset patients had spread to the brain during the follow-up duration. In contrast, the pituitary lesion of the other two adult-onset patients without panhypopituitarism and all the childhood-onset patients had not changed.

Conclusion

DI was the initial presentation symptom of HPA involvement. Anterior pituitary hormone deficiency followed in some patients. Compared with the childhood-onset patients, the adult-onset patients were more likely to have panhypopituitarism and a poor prognosis.

Figures and Tables

Fig. 1
Langerhans cell histiocytosis of the mastoid in a 52-year old man. A. This shows the typical morphology of an inflammatory infiltrate including eosinophils and histiocyte-like mononuclear cells having abundant amphophilic cytoplasm and grooved nuclei (H&E staining, × 400). B. Langerhans cells disclose strong immunoreactivity for S-100 (immunohistochemistry, × 200).
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Fig. 2
Different prognosis of pituitary lesion (sella MRI). A. Pituitary stalk mass is seen at baseline (Patient 3). B. Pituitary stalk mass increased after 2 years (Patient 3). C. Pituitary stalk mass is seen at baseline (Patient 4). D. Pituitary stalk mass had no change after 3 years (Patient 4). E. Pituitary stalk thickening is seen at baseline (Patient 5). F. Pituitary stalk thickening disappeared after 12 years (Patient 5).
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Table 1
Clinical characteristics, disease extent, endocrine involvement and treatment modality of the ten patients
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*Biopsy site. ACTH, adrenocorticotrophic hormone; DI, diabetes insipidus; GH, growth hormone; LH-FSH, luteinizing hormone-follicle stimulating hormone; PRL, prolactin; TSH, thyroid stimulating hormone.

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